Disorders of the Blood and

Blood Forming Organs

Introduction
This lesson provides an overview of the
assessment of the hematological system as a
context for diagnosis and treatment of common
hematologic problems, including neoplasms,
and for health promotion. e!ll cover the
following topics"
#
$istory
#
%hysical examination
#
&ommon screening and diagnostic tests
#
$ealth'promotion behaviors
$istory
Current complaint
# ()in" pruritus *intense, chronic, generali+ed itching,consider
$odg)in disease-
# .outh" bleeding gums, sore tongue, poor dentition
# &ardiovascular" shortness of breath, palpitations, fainting, or
paleness
#
/eurologic" gait disturbance, paresthesia, loss of taste, depression,
confusion, headache
# 0astrointestinal" dar) urine, decreased appetite, cravings for ice or
dirt *pica-, blood in stool, dar) stools
# $ypothyroid symptoms
#
.enses" fre1uency, duration, heaviness of menstrual flow
#
2nexplained bleeding
# %ain, particularly in the bone
# /ight sweats
# Fever
$istory
Medical history
#
Total or partial gastrectomy
# $ypothyroidism
# 3ecent infection
# &ancer or treatment with chemotherapeutic agents
#
4rthritis
Medication history
# 4ny medications ta)en regularly
# /onsteroidal antiinflammatory drugs */(4IDs-
Family history
#
4nemia
#
5aundice
# 0allstones
# Bleeding disorders
# &ancer
#
.other!s pregnancy history, if patient is a child
$istory
Psychosocial history
#
Tobacco use
#
4lcohol use
#
&ocaine use
#
6xposure to chemicals or heavy metals
#
6xposure to radiation
Dietary history
#
(trict vegetarianism *may cause vitamin B78
deficiency-
#
%redominately carbohydrate diet *may cause
folate deficiency-
#
hether formula is fortified with iron
#
9uantity of mil) consumed
%hysical 6xamination
Examination
# .easure height, weight, and body mass index *B.I-
# 4ssess general appearance and level of orientation
# &hec) the s)in for spider angiomas, vitiligo, pallor, petechiae, and
purpura
#
4ssess the condition of the nails
# :oo) for glossitis, angular stomatitis, eyelid edema, con;unctival
pallor, and retinal hemorrhage
# /ote the presence of lymphadenopathy greater than 8 cm
# 4ssess the pulmonary<cardiovascular system for systolic e;ection
murmur, (= sounds, tachycardia, tachypnea, angina, chronic heart
failure, and persistent cough
#
/ote loss of vibratory or position sense or presence of ataxia,
wea)ness, impaired thought processes, tremors, and cranial'nerve
palsies
# 4ssess the patient for ;aundice, hepatosplenomegaly *always an
abnormal finding-, ascites, and abdominal masses
%hysical 6xamination
Diagnostic procedures
# &omplete blood count, including mean corpuscular volume and red
blood cell distribution width
# (erum B78 determination
# Folate assay
#
3eticulocyte count
# Ferritin determination
# Total iron'binding capacity
# $emoglobin electrophoresis studies
#
Thyroid' and liver'function tests
#
Fecal occult blood test
# 3egular carcinoembryonic antigen testing in some cancer survivors
# %rostate'specific antigen test for prostate cancer
# &olonoscopy or upper or lower gastrointestinal series as indicated
#
Bone'marrow examination
#
.agnetic'resonance imaging or computed tomography as
appropriate
$ealth %romotion
#
4nemia screening at recommended intervals
#
Osteoporosis screening for oncology patients
#
3egular cancer screening, with particular
attention paid to the areas most at ris),
depending on the type of cancer
#
%neumococcal and influen+a vaccines for
cancer survivors
#
0enetic counseling to help determine familial
ris) and the need for regular testing
Introduction
4nemia is a condition in which the concentration of
hemoglobin or the number or volume of red
blood cells *3B&s- is reduced to a value that is
below normal. :eft untreated, it can result in
hypovolemic shoc), permanent neurologic
damage, and cardiac dysrhythmias.
#
In this lesson, we!ll cover the following types of
anemia"
#
Iron'deficiency anemia
#
Thalassemia
#
%ernicious anemia
Incidence
# 4nemia occurs most fre1uently in young children, women of
reproductive age, and older adults
#
Iron deficiency is the most common cause of anemia> the most li)ely
cause of diet deficiency in children, it is prevalent in approximately
=? of children ages 7 to @ years
# 4 low hemoglobin level is present in about A? of women ages 7@ to
BB years and is especially common during pregnancy
# The prevalence of anemia increases in people ages C@ years and
older
#
%rematurity is a predisposing factor in the early development of iron
deficiency
# &onsumption of cow!s mil) before the age of C months or, in older
infants or toddlers, a switch to whole cow!s mil), may lead to
nutritional iron deficiency> nonfortified formula may also pose a
problem
#
:ower socioeconomic status is a ris) factor
#
6thnicity plays a role
%athophysiology
#
4nemia may be caused by impaired production,
increased destruction, or rapid loss of 3B&s
#
The physiologic defects caused by anemia are a
decrease in the oxygen'carrying capacity of the
blood and a reduction in the oxygen available to
the tissues
#
The signs and symptoms of anemia are a result
of failure to oxygenate tissues and the degree of
acuity *gradual onset of anemia allows time for
compensatory mechanisms to increase
oxygenation-
#
4nemias can be classified on the basis of causal
mechanism or by 3B& structure
$istory
Current complaint
# Irritability or mood disturbances
# Dyspnea *particularly exertional-
# Fatigue and wea)ness
#
%alpitations
#
Di++iness
# $eadache
# 6dema
# 5aundice *particularly during the neonatal period, resulting from
hyperbilirubinemia-
#
Bleeding
#
3ecent weight gain or loss
# 4norexia
# /ausea or vomiting
# Decreased food inta)e
#
Diarrhea
# Bone pain
$istory
Medical history
#
4nemia with previous pregnancies
#
Infection
#
$eart murmur
#
&hronic illness
#
Drug use
#
%ast viral infections
#
6nvironmental exposures at home and in the wor)place
*e.g., lead, chemicals, heavy metals-
Family history
#
6thnic origin
#
Travel
#
0allstones or splenectomy *may signal a blood
dyscrasia-
$istory
Medication history
#
4nticonvulsants and chemotherapy
#
/onsteroidal antiinflammatory drugs */(4IDs-
Diet history
#
Decreased iron, folate, vitamin B78, or vitamin 6
inta)e
#
4lcohol ingestion
#
%ica
#
Formula type and amount
#
2se of cow!s mil)
%hysical 6xamination
Examination
#
0eneral appearance, including sensitivity to cold, weight loss,
lethargy, mood changes *indicating vitamin B78, folate, or iron
deficiency-
# 5aundice, bleeding, pallor, petechiae, or purpura
# 0lossitis *indication of vitamin B78, folate, or iron deficiency- or
angular stomatitis
# 6yelid edema, con;unctival pallor, retinal hemorrhage, color
blindness *indication of vitamin B78, folate, or iron deficiency-
#
Tachypnea
# %alpitations> dyspnea> tachycardia, systolic e;ection murmur, or
gallop> angina> myocardial infarction> congestive heart failure
# $epatosplenomegaly *most common in hemolytic anemia- or other
masses, lymphadenopathy, anorexia
#
Bone pain
#
/eurologic signs such as headache, vertigo, irritability, depression,
impaired thought processes, tremors, peripheral loss of sensation,
ataxia, and loss of taste and smell *most common in vitamin B78
deficiency-
%hysical 6xamination
Diagnostic procedures
# &omplete blood count *&B&-, including all indexes, mean cell volume
*.&D-, mean corpuscular hemoglobin *.&$-, and .&$ concentration
# $emoglobin and hematocrit assays
# %eripheral'blood smear *si+e and inclusions-
# 4bsolute reticulocyte count and bone'marrow testing
# Direct and indirect bilirubin assays
# :actate dehydrogenase, aspartate aminotransferase, uric acid, total iron,
vitamin B78, vitamin 6, and folic acid determinations
# Direct and indirect &oombs tests
# Total iron'binding capacity *TIB&-
# Ferritin assay *most useful test in differentiating iron'deficiency anemia from
anemia of chronic disease-
# Free erythrocyte protoporphyrin concentration
# (erum methylmalonic acid and homocystine levels *increased in vitamin
B78 deficiency-
# &omposite test results to help identify the type of anemia
# 3esults compared with age'appropriate standards
# 4ssessment of findings of gastrointestinal blood loss, hemolysis, nutritional
deficiencies, malignancy, chronic infection *such as subacute endocarditis-,
renal or hepatic disease, and other chronic disease
%hysical 6xamination
Differential diagnosis
#
4utoimmune pancytopenia
#
.arrow infiltration with a solid tumor
#
.arrow suppression caused by drug toxins or infections
#
Bleeding colitis
#
Osteomyelitis
#
%yelonephritis
#
Blood loss
#
$emolysis
#
&hronic inflammatory bowel disease
#
3heumatoid arthritis
#
(ystemic lupus erythematosus
#
:eu)emia
#
4ge'specific diagnosis
Treatment
Nonpharmacologic: iron-deficiency anemia
#
6valuate for upper'gastrointestinal bleeding, the
cause in 8E? to =E? of older adults
#
6valuate for bleeding in the colon> polyps and
cancer account for 7@? to =E? of patients
#
3ecommend sources of iron'fortified products,
including red meat and iron'fortified formula and
cereal
Nonpharmacologic: anemia of chronic disease
#
Treat the underlying cause
#
Iron therapy is not helpful
#
6rythropoietin may be helpful
Treatment
Nonpharmacologic: pernicious anemia
#
3ecommend a diet containing meat and dairy products
for vitamin B78 and folate deficiencies
#
&onsider oral B78 supplementation if patient is a vegan
or strict vegetarian
#
4dvise consumption of legumes, leafy green vegetables,
fruits, and liver to treat folate deficiency
Nonpharmacologic: thalassemia
#
In minor thalassemia, iron therapy is not helpful and
should be avoided
#
In ma;or thalassemia, transfusion may be ordered as
re1uired, as well as iron chelation
#
(plenectomy may be indicated in cases involving
hypersplenism
#
Bone'marrow transplant may be indicated
Treatment
Pharmacologic: iron-deficiency anemia
# The patient should ta)e @E to CE mg of elemental iron and avoid
ta)ing the tablet with mil)
# .etaboli+ation may be enhanced with orange ;uice or vitamin &
# Ingestion with meals diminishes gastrointestinal effects but also
decreases absorption
#
Discontinue therapy when the ferritin level is greater than @E mcg<:
# 3eticulocytosis is seen within a wee) of the initiation of oral iron
supplementation
# If side effects are a problem, decrease the dosage of iron
Pharmacologic: anemia of vitamin B! deficiency
#
4dminister 7EEE mcg of cyanocobalamin or hydroxocobalamin
subcutaneously each wee)
# 4lternatively, have the patient ta)e 8 mg of oral cyanocobalamin
*this is perhaps superior to subcutaneous administration-
Treatment
Pharmacologic: folate-deficiency anemia
#
%rescribe oral folic acid 7.E mg
Pharmacologic: anemia of chronic disease
#
The goal of treatment is to eradicate the anemia
and its cause
#
2se recombinant human erythropoietin
intravenously or subcutaneously @E to 7EE
units<)g three times a wee) to treat anemia of
renal failure or other anemia of chronic disease
#
2se oral prednisone *F7EE mg- to treat
autoimmune hemolytic anemia
6ducation
"ron-deficiency anemia
#
Identify causes of low iron and discuss the treatment plan
#
Teach the patient, caregiver, or family about ways to get a diet high
in iron
# 6xplain the side effects of iron therapy
# 6xplain the importance of screening for anemia once during infancy,
during pregnancy, and in adults older than @E years
Pernicious anemia
#
6xplain the cause and nature of the disease
# 6xplain the need for lifelong vitamin B78 replacement
# 3eview the side effects of vitamin B78 in;ection
#halassemia
#
4void iron replacement in minor cases
# 6ncourage prenatal and genetic counseling and 3h isoimmuni+ation
# 6xplain the importance of screening
Follow'2p
"ron-deficiency anemia
# In infants, chec) for an increased reticulocyte count in 8 to = days,
rechec) for normal hemoglobin in 8 to = wee)s, and continue iron
therapy for 8 to = months after the hemoglobin level has returned to
normal to replenish iron stores
# In adults, rechec) the absolute reticulocyte count in 7E days,
continue iron for = months after the return of normal hemoglobin to
replenish iron stores, and order another &B&
# 4n increase in hemoglobin of approximately 7 g<d: in 7 month
indicates successful treatment
Pernicious anemia
# (chedule a visit with the older adult or patient with a cardiac
condition after BG hours to prevent hypovolemia, which can result
from a rapid increase in 3B& production
#
&onsider iron supplementation
# &hec) the initial hematologic response in B to C wee)s, then every C
months for hematocrit, with a stool chec) for occult blood
Folic acid deficiency
# 3epeat the hemoglobin measurement and hematocrit in 8 to B
wee)s *expect an increase of 8 points in hemoglobin within 7
month-
3eferral
#
3efer the patient to the emergency
department if anemia is severe and the
patient is displaying symptoms
#
4 gastroenterology referral is appropriate
if a gastrointestinal cause of bleeding is
suspected
#
4 hematologist should see any patient
with a genetic anemia
#
The patient with nutritional iron'deficiency
anemia may benefit from a referral to a
dietitian
(ummary
#
ithout treatment, anemia can lead to
hypovolemic shoc), permanent neurologic
damage, and cardiac dysrhythmias.
Board 9uestions
# orldwide, what is the most common anemiaH
I %ernicious anemia
I Folate'deficiency anemia
I 4nemia of chronic disease
I Iron'deficiency anemia
.ost of the bodyJs iron is obtained from"
' 4nimal based food sources
' 3ecycled iron content from aged 3B&s
' 6ndoplasmic reticulum production
' Degetable'based food sources
hich of the following reasons accounts for why polycythemia can result in
a TI4H
'the tissue is resistant to insulin
'the blood is too thic), resulting in clotting
'the platelets are inhibited
'the blood is too thin, resulting in decreased profusion
hich of the following is most consistent with iron deficiency anemiaH
'low mean corpuscular volume *.&D-, normal mean
corpuscular hemoglobin *.&$-
'low .&D, low .&$
'low .&D, elevated .&$
'normal .&D, normal .&$
One of the earliest laboratory ma)ers in iron deficiency a anemia is"
'an increase in 3B& distribution width *3D-
'a reduced hemoglobin level
'a low .&$ level
'an increased platelet count
Oral iron is prescribed for a child with an iron deficiency anemia. The /%
instructs the mother to administer the iron"
';ust before a meal
';ust after a meal
'between meals
'with a fruit low in Dit. &
'
4 BG year old woman developed iron deficiency anemia after excessive
perimenopausal bleeding, successfully treated by endometrila ablation.
$er $ct level is 8@?, and she is ta)ing iron therapy. 4t @ days into
therapy you expect to find"
'a correction of the mean cell volume
'a 7E? increase in the $ct level
'bris) reticulocytosis
'a normal ferritin level
4 healthy =B year old man as)s whether he should ta)e an iron
supplement. Kou respond that"
'this is a prudent measure to ensure health
'Iron deficiency anemia is a common problem in men of his
age
'use of an iron supplement in the absence of a documented
deficiency can lead to an iatrogenic iron overload
'excess iron is easily excreted
hich of the following is the best advice on ta)ing ferrous sulfate to
enhance iron absorptionH
Lta)e with other medicationM
Lta)e on a full stomachM
Lta)e on an empty stomachM
Ldo not ta)e with Dit. &M
4 BE year old woman with pyelonephritis who is ta)ing ciprofloxacin
and is being treated for iron deficiency anemia with ferrous sulfate as)s
about ta)ing both medications. Kou advise that"
'she should ta)e the meds with a large glass of water
'an active drug compound is potentially formed if
the 8 meds are ta)en together
'she can ta)e the meds together to enhance
adherence to therapy
'the ferrous sulfate potentially slows 0I motility and results in
enhanced ciprofloxacin absorption
One month into therapy for pernicious anemia, you wish to chec) the efficacy of
the intervention. The best lab test to order at this point is a"
'(chilling test
'hemoglobin measurement
'reticuloycyte count
'serum ferritin
4 woman who is planning a pregnancy should increase her inta)e of which of
the following to minimi+e the ris) of neural tube defect in the fetusH
'iron
'niacin
'folic acid
'Dit. &
The /% is reviewing the lab results of a child with aplastic anemia, the B&
count is 8EEE<$: and the platelet count is 7@E,EEE<mmN. hich of the following
interventions will the /% incorporate into the plan of careH
-maintain strict isolation precautions
-encourage the child to use a soft toothbrush
-avoid unnecessary in;ections
'encourage 1uiet play activities
3is) factors for folate deficiency anemia includes"
'mennorhagia
'chronic ingestion of overcoo)ed foods
'use of nonsteroidal anti'inflammatory drugs
'gastric atrophy
Folate deficiency anemia causes which of the following changes in the 3B&
indicesH
'microcytic, normochromic
'normocytic, normochromic
'microcytic, hypochromic
'macrocytic, normochromic
4 patient ta)ing ferrous sulfate for iron deficiency anemia is complaining of
nausea and vomiting. hat should the /% suggest to address the problemH
'ta)e the iron with orange ;uice
'discontinue the iron
'ta)e the iron BID instead of TID
'ta)e iron on an empty stomach
%ernicious anemia is usually caused by"
'dietary deficiency of vitamin B78
'lac) of production of intrinsic factor by gastric
mucosa
'3B& en+yme deficiency
'a combination of micronutrient deficiencies caused
by malabsorption
%ernicious anemia causes which of the following changes
in the 3B& indicesH
'microcytic, normochromic
'normocytic, normochromic
'microcytic, hypochromic
'macrocytic, normochromic
&ommon physical examination findings in patients with pernicious
anemia include"
'hypoactive bowel sounds
'stoc)ing'glove neuropathy
'thin, spoon'shaped nails
'retinal hemorrages
Kou examine a BO year old man with the following results on
hemogram"
$gb P 7@ g
$ct P B@?
.&D P 7EG f:
These values are consistent with"
'pernicious anemia
'alcohol abuse
'thalassemia minor
'Fanconi disease
Kou examine a 88 year old 4sian woman. (he has no presenting
complaint. $emogram results are as follows" $gb A.7 *normal 78 to 7B
g-> $ct 8G? *normal =C? to B8?-> 3BD @ million *normal> =.8 to B.=
million-> .&D CGf: *normal GE to ACf:-> 3B& distribution width *3D-
7=? *normal Q7@?-. The most li)ely diagnosis is"
'iron deficiency anemia
'&ooley anemia
'alpha thalassemia minor
'hemoglobin Bart
4 CG year old man is usually healthy but presents with new onset of
Lhuffing and puffingM with exercise. %6 reveals con;unctiva pallor and a
hemic murmur. $gb is O.C g> .&D is O f:. The most li)ely clinical
problem is"
'poor nutrition
'occult blood loss
'malabsorption
'microcytosis

Kou examine a @O year old woman with rheumatoid arthritis and you
find the following results on hemogram"
$gb P 7E.@
$ct P ==?
.&D P GGf:
The lab findings are most consistent with"
'pernicious anemia
'anemia of chronic disease
'beta thalassemia minor
'folate deficiency anemia
Kou examine a 8O year old woman with menorrhagia and not the
following results on hemogram"
$gb P 7E.7
$ct P ==?
.&D P O8 f:
%hysical exam is li)ely to include"
'con;unctiva pallor
'hemic pallor
'tachycardia
'no specific anemia related findings
3esults of hemogram in chronic disease include"
'microcytosis
'anisocytosis
'reticulocytopenia
'macrocytosis
4 @B year old white male is being evaluated because he was found to have a low $gb
*7E.8- and $ct. $e tells you that except for some fatigue and a @ to C pound weight loss
over the past = months, he has not felt ill. Kou repeat the &B& and confirm that the
patient has microcytic, hypochromic anemia. Kou suspect iron defficiency. 4t this point,
which of the following would be your best course of actionH
'administer a &406 1uestionnaire, since you suspect alcohol related
gastritis
'admit the patient to the hospital for blood transfusion
'obtain more history and perform a physical exam including a D36 and test for
occult blood
'start oral ferrous sulfate and have the patient return to see you in B to C wee)s
for a repeat &B&

hen prescribing erythropoietin supplementation, the /% considers
that"
'the adrenal glands are an endogenous source
'the addition of iron and other micronutrient supplementation is
advisable
'its use is as an ad;unct in treating thrombocytopenia
'with its use, the 3B& life span is prolonged
In the first wee)s of pernicious anemia therapy with parenteral vitamin
B78 in a CG year old women, the patient should be carefully monitored
for"
'hypernatremia
'dehydration
'hypo)alemia
'acidemia
hich of the following conditions is unli)ely to result in
anemia of chronic diseaseH
'rheumatoid arthritis
'peripheral vascular disease
'chronic renal insufficiency
'chronic osteomyelitis
In health, the ratio of $gb to $ct is usually"
'7"7
'7"8
'7"=
'7"B
Introduction
#
(ic)le cell anemia is an abnormality of the globin
genes *i.e., a hemoglobin disorder-. This
condition is characteri+ed by severe chronic
hemolytic disease resulting from premature
destruction of the brittle, deformable
erythrocytes. :eft untreated, sic)le cell anemia
can result in tissue ischemia and infarction that
can damage virtually every organ system. In this
lesson, we!ll review the presentation, diagnosis,
and management of sic)le cell anemia
Incidence
#
The most common single genetic disorder in
blac)s
#
4ffects more than OE,EEE blac)s, a third of
whom are between the ages of 8 and 7C years
#
4ffects one in BEE blac) infants
#
G? of blac)s are hetero+ygous carriers of the
sic)le cell gene and have the trait
#
(ic)le cell trait and anemia affect people in
many parts of 4frica, the .editerranean, and
parts of Tur)ey, the .iddle 6ast, and India
%athophysiology
# R$emoglobinopathyR might be a better term than RanemiaR> in sic)le
cell anemia, hemoglobin is sub;ect to a structural change in the
amino acid se1uences of the globin chains
#
Both parents must have sic)le hemoglobin for their child to have
sic)le cell anemia> only one ( is present in the sic)le cell trait
# (ic)le hemoglobin differs from normal adult hemoglobin in the
replacement of a glutamic acid at the sixth position of its amino acid
chains with valine
# In the oxygenated state, the sic)led hemoglobin functions normally
#
hen this hemoglobin is deoxygenated, an interaction occurs that
causes the formation of polymers that elongate to form rigid
crystalline rods
# Other symptoms of sic)le cell anemia are caused by the ischemic
changes resulting from vascular occlusion caused by high numbers
of sic)led red blood cells *3B&s-, referred to as RcrisesR
# (ic)led 3B&s circulate differently than do normal 3B&s> their
effects are different in newborns, young children, older children, and
adults
$istory
Current complaint
#
Irritability
#
Dyspnea
#
Fatigue
#
%alpitations
#
$eadache
#
6dema
#
5aundice
#
Bleeding
#
%allor
#
Infection
#
$eart murmur
#
&hronic illness
#
Bone pain
$istory
Medical history
#
Diral infections
#
$ospitali+ations
Medication history
#
4ll drugs, but particularly anticonvulsants
Family history
#
4frican, Tur)ish, .iddle 6astern, Indian, or
.editerranean ancestry
#
(ic)le cell anemia
#
%rior testing for sic)le cell trait or disease
%hysical 6xamination
Examination
#
Dital signs, weight, and height
#
0eneral appearance, including sensitivity to cold, weight
loss, and lethargy
#
5aundice, bleeding, pallor, petechiae, purpura
#
0lossitis or angular stomatitis
#
6yelid edema or retinal hemorrhage
#
Tachypnea
#
Tachycardia, murmur, or gallop> angina> myocardial
infarction *.I-> congestive heart failure
#
$epatosplenomegaly or other masses,
lymphadenopathy, anorexia *chec) the si+e of the spleen
during every examination-
#
Bone pain or leg ulcers
#
/eurologic symptoms, including vertigo, irritability,
depression, and impaired thought processes
%hysical 6xamination
Diagnostic procedures
#
/ewborn screening *re1uired in B7 states and the District
of &olumbia-
#
&omplete blood count, including all indexes, mean
corpuscular volume, mean corpuscular hemoglobin, and
mean corpuscular hemoglobin concentration
#
Blood smear *typically reveals target cells, poi)ilocytes,
and irreversibly sic)led cells-
#
6rythrocyte sedimentation rate *slow-
#
:iver'function testing *abnormal findings-
#
Bilirubin assay *increased-
#
Bone marrow *may be hyperplastic, with erythroid
predominance-
#
$emoglobin studies, including electrophoresis to screen
for sic)le cell *hemoglobin ((- and sic)le cell trait
%hysical 6xamination
Differential diagnosis
#
3heumatic fever
#
3heumatoid arthritis
#
Osteomyelitis
#
:eu)emia
Treatment
The goal of treatment is to provide early diagnosis, comprehensive
medical care, and prophylactic care to reduce episodes of infection
and complications.
Nonpharmacologic
# 6valuate any illness involving a temperature of =G.@S & or higher
through the use of blood cultures and careful observation
# Transfuse 3B&s to improve oxygen'carrying capacity during crises
*commonly indicated by stro)e-
Pharmacologic
#
For illnesses involving fever, administer intravenous broad'spectrum
antibiotics
# %rescribe folic acid to ensure ade1uate amounts for the high
erythrocyte turnover
# 3ecommend prophylactic penicillin starting by the age of 8 months
through at least C years
#
4dminister a pneumococcal vaccine and a yearly influen+a vaccine
#
4dminister pain medications in times of crisis
6ducation
#
6ncourage sic)le cell screening in parents
before pregnancy
#
Identify causes of sic)le cell crisis and disease
process
#
6xplain precipitating factors of sic)le cell crises
#
6mphasi+e the need for routine health care
#
Offer genetic counseling to all adolescents, as
well as family'planning information
#
%rovide psychosocial support
Follow'2p and 3eferral
Follo%-up
#
(ee affected children every = months until they are 8
years old, then every C months until age @, and then
yearly
#
Order periodic urinalyses to chec) for early signs of renal
disease
#
%erform retinal examinations after age 7E to rule out
retinal disease
#
Order an ultrasound to detect gallbladder disease if the
patient complains of pain in the right upper 1uadrant
&eferral
#
3efer the patient to a hematologist for multidisciplinary
approach and pain management
(ummary
#
ithout treatment, sic)le cell anemia can
lead to tissue ischemia and infarction that
can damage virtually every organ system.
Introduction
In this lesson, we!ll cover the presentation,
diagnosis, and management of the
following bleeding disorders"
#
%urpura
#
%latelet disorders
#
&oagulation'factor deficiencies
Incidence
'enoch-(ch)nlein purpura
#
Ig4'mediated systemic vasculitis of small blood
vessels that causes palpable
nonthrombocytopenic purpura, abdominal pain,
arthritis, and nephritis
#
.ainly affects boys between the ages of 8 and
77 years
#
4ffects 7B of 7EE,EEE individuals
#
%receded by upper'respiratory infection *23I-,
with (treptococcus the most common causative
organism
#
Involves the renal system in GE? of patients
#
:asts B to C wee)s in most patients
#
Treatment is supportive
Incidence
von *ille+rand disease
#
4utosomal disorder *chromosome on gene 78- that
produces a defect of factor DIII procoagulant activity
resulting from decreased von illebrand factor
#
4ffects men and women e1ually
#
%resents as menorrhagia, epistaxis, or gingival
hemorrhage
'emophilia
#
Three types" hemophilia 4 *classic hemophilia-,
hemophilia B *&hristmas disease-, and hemophilia &
#
4ffects men almost exclusively
#
OE? of patients are positive for human
immunodeficiency virus *$ID-
#
(ymptoms include hot, swollen, painful ;oints
Incidence
"diopathic throm+ocytopenic purpura ,"#P-
#
Increased destruction of platelets
#
Often follows an infection by 8 to = wee)s
#
4utoimmune disease
#
&haracteri+ed by development of Ig0
autoantibodies for platelets
#
%ea) occurrence between 8 and B years
#
4ffects men and women e1ually
#
4pproximately GE? of children will experience
remission within a few wee)s
#
In adults, IT% tends to be chronic, with few going
into remission
%athophysiology
#
Bleeding disorders are deviations from the
normal clotting cascade
#
%latelet disorders are caused by decreased
production of platelets, increased destruction of
platelets, and se1uestration of platelets
#
&oagulation factor deficiencies involve factors
DIII, IT, and TI> and von illebrand factor
#
%urpura is described on the basis of si+e
*petechiae, purpura, ecchymosis-
$istory
Current complaint
#
4symmetrical bruising of acute onset
#
%etechiae and purpura *uncommon in hemophilia-
#
4cute phase lasting 7 to 8 wee)s
#
/osebleeds that are difficult to control
#
Bleeding from circumcision
#
Bleeding with tooth eruption
#
4bdominal pain
#
&linically well appearance, no pallor or enlarged lymph
nodes, and no complaints of fever, tiredness, weight
loss, or bone or ;oint pain
Medical history
#
(ystemic lupus erythematosus
#
3ecent infection
#
$ID infection *often a result of the need for blood'
products transfusions-
$istory
Family history
#
Bleeding disorders
Medication history
#
Drugs whose effects mimic IT%" penicillin,
valproic acid *Depa)ene-, 1uinidine,
1uinine, sulfonamides, cimetidine
*Tagamet-, heparin
Psychosocial history
#
Occupational exposure to chemicals
%hysical 6xamination
&onduct a comprehensive physical examination,
paying special attention to particular areas that
may reveal a bleeding disorder or the cause of
one.
Examination
#
:oo) for a generali+ed petechial rash,
asymmetrical on the legs *IT%, sudden onset in
children and gradual onset in adults-
#
&hec) for purpura on the legs *$enoch'
(chUnlein purpura-
#
/ote absence of hepatosplenomegaly and pallor
#
4ssess patient for hemarthrosis *hemophilia-
%hysical 6xamination
Diagnostic procedures
#
&omplete blood count *platelet count decreased to fewer
than @E,EEE cells<mm=, with enlarged platelets, in IT%-
#
%rothrombin time *%T> normal in hemophilia-
#
4ctivated partial thromboplastin time *a%TT> prolonged in
hemophilia-
#
Bone'marrow aspiration for definitive diagnosis of IT%
#
4ssessment of coagulation parameters *in hemophilia,
factor DIII is reduced> %T, fibrinogen, and bleeding time
are normal-
#
In hemophilia, coagulation'factor activity of less than 8
units<d: indicates severe disease with spontaneous
bleeding
%hysical 6xamination
Differential diagnosis
#
(ystemic lupus erythematosus
#
$ID infection
Treatment
# If bruising is mild and patient shows no evidence of mucous'membrane
bleeding, no treatment is prescribed
# If the chance of bleeding is high *platelets Q =E,EEE cells<mm=-, the patient
may re1uire hospitali+ation
# 4void administration of intramuscular in;ections, aspirin, and nonsteroidal
antiinflammatory drugs
# 4dminister prednisone 7 to 8 mg<)g daily for IT% until the platelet count is
normal, then slowly taper the dosage
# &onsider using high'dose Ig0 or intravenous glucocorticoids when the
platelet count is less than 8EE,EEE cells<mm= or if significant bleeding
occurs
# &onsider splenectomy in the adult patient with a platelet count of less than
=E,EEE cells<mm= after C wee)s of treatment and in children after 7 year of
treatment
# Transfusion of platelets or coagulation factors may be re1uired
# In hemophilia 4, replace factor DIII and consider implantation of genetically
altered fibroblasts for severe hemophilia
# In hemophilia B, replace factor IT and administer cyclophosphamide and
prednisone
# %rescribe steroid therapy if intracranial bleeding is severe or the platelet
count is very low
# %erform regular assays for $ID and hepatitis 4, B, and &
# 4dminister hepatitis 4 and B vaccinations as soon as possible
6ducation
#
Teach the patient about bleeding disorders, signs and symptoms
related to the site of bleeding, recommended treatments, and
treatment complications
# 6xplain administration and storage of factor'replacement products
# Teach parents how to perform home infusion, and, later, teach
children self'infusion methods
#
Tell patients to avoid contact sports, ta)e temperature orally rather
than rectally, and avoid sharp foods such as tortilla chips
# Tell patients to refrain from using ibuprofen, aspirin, and aspirin'
containing products such as %epto'Bismol
# (uggest the use of a soft'bristle toothbrush or washcloth and
mouthwash to maintain oral hygiene and minimi+e bleeding
# Teach parents and children how to stop nosebleeds by sitting
upright and pinching the nostrils closed for at least 7E minutes
#
3ecommend that patients with IT% avoid prolonged exposure to
sunlight to prevent petechiae and purpura
# (uggest the use of a padded crib and playpen for the infant with a
bleeding disorder
# 4dvise parents or caregivers to notify the school nurse of the child!s
diagnosis and treatment and any special considerations
Follow'2p and 3eferral
Follo%-up
#
Follow up as recommended by the hemophilia
treatment center *$T&-> treatment is managed
at the $T& in a multidisciplinary approach
#
%erform regular assays for $ID and hepatitis 4,
B, and &
&eferral
#
3efer the family for genetic counseling at $T&
#
3efer the patient to a home health'care agency
that can perform infusions
#
3efer the patient and family to the /ational
$emophilia Foundation
Board 9uestion
The /% student is caring for a child with hemophilia. The
preceptor reviews the plan of care and as)s the student
to describe the characteristics of this disorder. hich
statement by the student indicates a need for further
student learningH
'hemophilia is inherited in a recessive manner via a genetic
defect on the T chromosome
'males inherit the carrier status from their fathers
'females inherit the carrier status from their fathers
'hemophilia 4 results from deficiency of factor DIII
:ead %oisoning
Introduction
#
:ead poisoning is defined as a blood lead
level greater than 7E mg<d:. :ead
poisoning can result in such complications
as mild to severe chronic anemia, mental
retardation or sei+ures, learning
disabilities, behavior and sleep disorders,
and developmental delays.
#
In this lesson, we!ll review the
presentation, diagnosis, and management
of lead poisoning.
Incidence
#
:ead poisoning is primarily a problem for children younger than age
C years because of the increased absorption *V@E?- of lead to
which they are exposed> children ages C to =C months are at
greatest ris)
# .ore than B? of children in the 2nited (tates have lead levels
higher than 7E mg<d:
#
.ore than 8@? of children live in housing with lead'based paint
#
4dults are generally exposed as a result of occupational exposure
# :ow blood hemoglobin, hematocrit, and iron increase ris)
# %ica is a ris) factor
# %oor diet increases the li)elihood of lead poisoning, as does poor
handwashing
#
Blac) children, children in urban areas, and children from low'
income families *those who 1ualify for .edicaid- are at greater ris)
of lead poisoning than other children are
%athophysiology
#
:ead is absorbed primarily through the respiratory and
gastrointestinal tracts
# 4fter lead is absorbed into the bloodstream, most of it is bound to
red blood cells *3B&s-
# The highest concentrations of lead are found in the liver, bone,
teeth, lungs, brain, )idneys, and spleen
# :ead is excreted very slowly from the body *biological half'life of 7E
years-> it has a half'life of =@ days in blood, BE days in soft tissue,
and 8E to =E years in bone
# :ead is substituted for calcium in the bone matrix> this lead is a
source for remobili+ation and continued toxicity
# :ead is a protean poison
# &hildren absorb approximately @E? of the lead they ingest, whereas
adults absorb only 7E? of the lead they ingest
#
(ources and pathways of lead exposure include lead'based paint,
pica, soil and dust, drin)ing water, parental occupation or hobbies,
food, and other sources
$istory
Current complaint
#
Inability to concentrate, unusual sleep patterns, poor
appetite, coordination problems, and other clinical signs
and symptoms *however, most children with lead
poisoning will be asymptomatic-
#
.outhing activities, including pica behaviors, use of a
pacifier, bottle drin)ing, or thumb'suc)ing
Medical history
#
%ast blood lead levels
#
4nemia
#
Developmental delay, particularly with respect to
language
#
0astrointestinal, hematologic, renal, reproductive, and
neurologic problems
$istory
Medication history
# Ditamins
# %rescription and nonprescription drugs
# Fol) remedies
Family history
#
:ead poisoning
# Psychosocial history
# Iron and calcium inta)e
# Type and amount of formula
#
Fre1uency of meals
#
4mount of fat in diet *a high'fat diet is associated with increased
lead absorption-
# 2se of pottery
# $obbies
#
Occupation *parents! occupations, if the patient is a child-
#
4ge and location of home, condition of paint, recent remodeling or
restoration, pipes
%hysical 6xamination
&onduct a comprehensive physical examination,
paying special attention to areas that may be
indicative of lead poisoning. Because symptoms
usually do not occur until the child has incurred
significant lead exposure, treat the presence of
any symptoms as a medical emergency.
Examination
#
0eneral appearance
#
Dital signs
#
0rowth parameters, including height, weight,
and head circumference
#
%sychosocial s)ills, cognition, language, and
behavior
%hysical 6xamination
Diagnostic procedures
#
(creening of high'ris) children by C
months of age
#
Blood lead determination, preferably with
the use of a venous sample
#
Obtain a radiograph of the )idneys and
upper bladder *paint chips in abdomen are
considered a positive finding-
#
:ong'bone radiographs
#
$emoglobin assay
%hysical 6xamination
Differential diagnosis
#
4ttention'deficit disorder
#
:earning disabilities
#
&entral nervous system tumor
#
4nemia
#
Developmental delay
#
&olic
#
.etabolic disorders
Treatment
#
Identify and eliminate sources of lead in the child!s
environment
#
If abdominal radiography confirms ingestion of
particulate lead, perform bowel decontamination as
indicated
#
Begin chelation therapy *class ID toxicity and higher-
with succimer or another common chelating agent
#
4dmit children with class D poisoning to the hospital for
treatment with ethylenediaminetetraacetic acid *6DT4- if
symptoms noted, the child is younger than age = years,
and the child has a lead level greater than CE mg<d:
#
In class D patients, treatment options include chelation
therapy with 6DT4, dimercaprol and 6DT4, or
dimercaptosuccinic acid
6ducation
#
6ncourage parents and caregivers to help prevent lead poisoning by
inspecting homes built before 7AOG for lead paint and having
children screened regularly for lead exposure
# If a home containing lead paint is to be repainted, stress that the
parents or caregivers should never try to do the ;ob themselves
because sanding and scraping tend to put more lead in the air>
encourage them to hire a professional
#
6xplain the factors that increase a child!s susceptibility to lead
exposure
# 6xplain the lead'level RreboundR phenomenon *in which lead moves
from storage in the bones bac) into the circulating blood- so that
parents don!t become discouraged when lead levels rebound
# Teach parents to provide a low'fat, high'calcium, iron'rich diet for
their children to reduce absorption and retention of lead
#
%rovide tips for cleaning, including wet'mopping floors with a
solution containing trisodium phosphate, contained in most
dishwashing detergents
# Discuss ways to protect children from lead ha+ards, such as
discouraging hand'to'mouth behaviors
# (tress the importance of fre1uent handwashing
Follow'2p
#
(chedule follow'up visits to loo) for sources of
lead exposure and conduct lead screening in
accordance with recommended schedules
#
4lthough lead levels drop to less than 8E mg<d:
within a few months of chelating treatment,
remember that some children with higher levels
of lead may ta)e much longer and need to
undergo chelation several times
#
&onduct follow'up testing on children receiving
succimer 7 wee) after the start of the regimen
and on completion of the 7A'day course of
treatment
3eferral
#
3efer cases of occupational lead poisoning and
children with class IIb or worse lead poisoning to
the local health department for environmental
investigation
#
3efer parents to a lead'poisoning program, if
available
#
3efer all patients with blood lead levels higher
than BB mg<d: to the local health department
#
3efer any child with an increased lead level who
exhibits symptoms of poisoning to a
hematologist or lead specialty clinic
#
&onsult with the appropriate health professional
in treatment of language, behavior,
developmental delay, and attention disorders
Board 9uestions
hich of the following is most li)ely to have lead poisoningH
'a developmentally disabled @ year old child who lives I a 7@
year old house on poor repair
'an infant who lives in a @ year old home with copper plumbing
'a toddler with lives in an G@ year old home
'a preschooler who lives near an electric generating plant
Kou are devising a plan to screen preschoolers for lead poisoning. The
most sensitive component of this campaign is"
'environmental history
'physical examination
'$ct level
'$gb level
%atients with plumbism present with which )ind of anemiaH
'macrocytic, hyperchromic
'normocytic, normochromic
'hemolytic
'microcytic, hypochromic
4t which of the following ages should screening begin for a
child who has significant ris) of lead poisoningH
'= months
'Cmonths
'7 year
'8 years
Intervention for a child with a lead level of 7E to 8E mcg<d:, usually
includes all of the following except"
'removal from the lead source
'iron supplementation
'chelation therapy
'encouraging a diet high in Dit. &
Intervention for a child with a lead level of BE to @E mcg<d:, ususally
includes"
'chelation therapy
'calcium supplementation
'exchange transfusion
'iron depletion therapy
Introduction
#
:ymphadenopathy is a condition involving a painless
lymph node> lymphoma is a malignant disease of the
lymphoid tissue, either $odg)in or non'$odg)in
lymphoma. :eu)emia is a proliferation of immature white
blood cells *B&s- originating in the bone marrow. (olid
tumors account for significant morbidity and mortality in
childhood. 4ll three types of cancer can result in the
development of other cancers or even death.
#
In this lesson, we!ll review the following topics related to
leu)emia, lymphoma, and solid tumors"
#
$istory
#
%hysical examination
#
&ommon screening and diagnostic tests
#
6ducation focused on preventing or minimi+ing the
effects of these diseases
:eu)emia" Incidence
.vervie%
#
&hildhood cancers occur at a slightly higher rate in males and at a
significantly higher rate in whites
# 4cute lymphoblastic leu)emia is one of the most common types of
childhood cancers
# Incidence of all types of leu)emia is approximately 7= cases in
7EE,EEE population per year
#
In general, men are affected more than women and whites more
than blac)s
# The prognosis is poorer for blac)s and for children younger than
age 8 years or older than age 7E years
# 0enetic predisposition increases ris)
# 4dvanced maternal age or history of fetal loss increases the ris) of
4.:
#
6nvironmental factors contribute to ris)
# Diral infections can increase ris)
# Immunodeficiency increases the li)elihood of leu)emia
:eu)emia" Incidence
#
/cute lymphocytic leu0emia ,/11-
#
4bnormal proliferation of immature lymphocytes
in the bone marrow
#
4ccounts for O@? to GE? of all childhood
leu)emias
#
4pproximately B cases per 7EE,EEE children
every year
#
4pproximately =@EE new cases each year in the
2nited (tates
#
%ea) incidence is = to @ years
#
.ale patients outnumber female patients by
7.="7
:eu)emia" Incidence
/cute myelogenous leu0emia ,/M1-
#
4bnormal proliferation of immature myeloid stem cells in
the bone marrow
#
3atio of 4.: to 4:: is 7"B
#
4ccounts for 7@? to 8@? of childhood leu)emia
#
&hild 4.: occurs before 8 years of age in approximately
7E? of patients
#
3elatively constant incidence from birth through
adolescence
Chronic lymphocytic leu0emia ,C11-
#
%ersistent absolute lymphocytosis for at least = months
#
.ost common type of leu)emia in the 2nited (tates
#
.ale patients outnumber female patients by 8"7 to ="7
#
4pproximately O@? of cases occur in people CE years
and older
:eu)emia" Incidence
Chronic myelogenous leu0emia ,CM1-
#
4bnormal proliferation of differentiating myeloid
cells in blood and bone marrow
#
Two types are ;uvenile and adult
1eu0emia in pregnancy
#
Fewer than one case in 7EE,EEE pregnancies
annually
#
:imited studies
#
In O8 cases of leu)emia studied between 7AO@
and 7AGG, C7? were 4.:, 8G? were 4::, and
O? were &.:
:eu)emia" %athophysiology
#
The overproliferation of immature B&s in the
bone marrow can replace completely the normal
bone marrow precursors, leading to a decrease
in red blood cells *3B&s-, platelets, and
granulocytes
#
:eu)emic cells also may proliferate in other
reticuloendothelial tissues and in other
extramedullary sites, such as the central
nervous system, testes, bones, or s)in
#
The cause is theori+ed to be ecogenetic, where
a genetic transformation of the progenitor *stem-
cell occurs in response to environmental agents
:eu)emia" $istory
Current complaint
#
In acute leu)emias, symptoms are present 7 to 8 wee)s before
diagnosis
# In chronic leu)emias, symptoms are chronic and less obvious
# (igns and symptoms of anemia are present at diagnosis in most
cases
#
(igns and symptoms of thrombocytopenia are present in O@? of
cases of childhood leu)emia
#
&entral nervous system *&/(- signs and symptoms are present in
less than 7E? of cases
# Bone pain is present at diagnosis in approximately 8=? of cases
# 0astrointestinal signs and symptoms result from proliferation of
leu)emic cells in the abdominal viscera, liver, and spleen
#
0enerali+ed lymphadenopathy *Q8 cm- is common at diagnosis as
a result of infiltration by leu)emic cells
:eu)emia" $istory
Current complaint
#
In male patients, testicular 4:: is manifested as a painless,
firm, unilateral or bilateral enlargement, with or without
discoloration
#
%allor, bleeding, fever, and pain result from bone'marrow
infiltration and failure *4:: and 4.:-
#
(plenomegaly, hepatomegaly, fever, night sweats, pallor,
weight loss, bone pain, and lymphadenopathy may indicate
&.:
#
&utaneous lesions, generali+ed lymphadenopathy, mar)ed
splenomegaly, hepatomegaly, hemorrhagic problems, facial
rash, and respiratory symptoms may indicate ;uvenile &.:
#
.alaise, increased fatigability, weight loss, anemia,
generali+ed lymphadenopathy, splenomegaly, and,as
disease progresses,hypogammaglobulinemia may indicate
&::
#
/eutropenia, fever, persistent infections, and abscesses are
other common complaints
:eu)emia" $istory
Medical history
#
6xposure to $T:D'I virus
#
3ecent viral illness
# %revious illnesses or surgery
# 4llergies
# Immuni+ations and screening tests
Medication history
#
6xposure to cytotoxic drugs or chloramphenicol
# .edications
# Tobacco, alcohol, or drug use
Family history
#
0enetic abnormalities
# &ancer
Psychosocial history
# 3elationships with family and friends
# 6xposure to ben+ene
:eu)emia" %hysical 6xamination
Examination
# Dital signs
# eight
# 0eneral appearance
#
(igns and symptoms of infection
#
6xcessive bruising, petechiae, pallor, rashes, infections, or leu)emia
cutis *leu)emic s)in infiltration, associated mostly with 4.:-
# /ec), axilla, supraclavicular, infraclavicular, epitrochlear, and
inguinal lymph'node enlargement
# $ead, eyes, ears, nose, and throat" fundi or papilledema
#
.urmurs caused by anemia or whee+ing and decreased breath
sounds resulting from a possible mediastinal mass
#
$epatosplenomegaly
# 2nilateral or bilateral firm testes or painless enlargement, with or
without scrotal discoloration
:eu)emia" %hysical 6xamination
Diagnostic procedures
#
&omplete blood count *&B&- with differential *the initial B& count
is the single most important predictor of prognosis-
# $emoglobin *Q7E g<d:> prognosis is poorer when this parameter is
low-
# Bone'marrow aspiration *8@? blasts-
#
&ytogenetic study, immunophenotyping, and special staining to
differentiate the type of leu)emia
# Bone'marrow biopsy *increased leu)ocytes or platelet precursors-
# :umbar puncture to rule out &/( involvement
# &hest radiography to chec) for mediastinal mass
#
(erum immunoglobulin levels *low at diagnosis in =E? of patients
with 4::-
#
(erum uric acid determination *increased level can lead to renal
failure-
# :iver'function tests *concentrations are increased-
# &alcium phosphate assay *calcium phosphate is increased-
#
Blood cultures *if patient is febrile-
:eu)emia" %hysical 6xamination
Differential diagnosis
#
4plastic anemia
#
.arrow infiltration with a solid tumor
#
4utoimmune pancytopenia
#
Immune thrombocytopenia
#
(evere megaloblastic anemia
#
Overwhelming infection
#
3heumatoid arthritis
#
.arrow suppression related to drugs, toxins, or
infections
#
In children, the most li)ely diagnosis is 4::, then 4.:
#
In adults, the most li)ely diagnosis is &.: or &::
#
In a pregnant patient, the most li)ely diagnosis is 4.:,
then 4::> the least li)ely is &.:
:eu)emia" Treatment
The goal of treatment is to eradicate leu)emic blast cells so that normal
cells can regrow.
/11
# &ombination chemotherapy, often in accordance with specific
multicenter protocols
# &ranial'spinal radiation is used in cases of &/( involvement
#
Treatment lasts 8 years plus a few months in girls and = years plus
a few months in boys
/M1
# 6xtremely intense combination chemotherapy
# Duration of treatment is shorter than that for 4::
#
&ranial'spinal radiation is used if &/( involvement is found
#
Bone'marrow transplantation *B.T- is performed if a complete
match is found
# .ost treatment is performed in the hospital
:eu)emia" Treatment
2uvenile CM1
#
0enerally resistant to therapy
#
.edian survival is less than A months from diagnosis
# B.T is the only hope of cure
/dult CM1
# 0oal of treatment is to provide symptomatic relief by lowering the
B& count and reducing liver and spleen si+e
#
$ydroxyurea or busulfan can be used to regulate leu)ocytosis
# B.T is the only possible cure
C11
# (table, symptom'free patients with or without lymphadenopathy or
splenomegaly do not re1uire treatment
#
(ymptoms are treated with al)ylating agents *e.g., chlorambucil-
and prednisone
:eu)emia" Treatment
1eu0emia in pregnancy
#
4.: or 4:: re1uires immediate and aggressive
treatment with combination chemotherapy
#
If 4.: or 4:: is diagnosed in the first trimester,
the prognosis for both mother and child is
extremely poor
#
If 4.: or 4:: is diagnosed and treated in the
second or third trimester, there is a O@? chance
of remission and only a 7.@? incidence of
congenital anomalies
#
&.: has a slow, indolent course, and for this
reason it may be treated after delivery> it may
also be treated during pregnancy with 0leevec
*imatinib mesylate-
:eu)emia" 6ducation
# 6xplain that avoidance of ris) factors may help prevent leu)emia,
but stress that there is no guarantee of this
#
6xplain the diagnosis and treatment plan, including expected and
toxic side effects of chemotherapy, if applicable
# %rovide clear supportive'care guidelines for the family, other
caregivers, and primary'care provider
# 6xplain medication and immuni+ation guidelines
#
6ncourage the patient to eat a healthy diet and exercise regularly
#
arn against the use of rectal thermometers, suppositories, and
tampons
# 3ecommend the use of a soft toothbrush or toothettes for oral care
and, if a central line is in place, antibiotic prophylaxis before dental
wor)
# 6ncourage the patient to limit sun exposure, stop tobacco use, and
avoid certain chemicals
#
Tell the patient and family to call for a &B& with differential if the
patient becomes febrile to rule out fever from neutropenia
:eu)emia" Follow'2p
#
3egular follow'up by a specialist should
be ensured, especially for children who
are cancer survivors, starting 8 years after
treatment is complete
#
Weep a summary of cancer treatment
#
(chedule regular dental follow'up visits,
especially if the child underwent treatment
before the age of @ years
#
(creen the patient regularly for depression
#
(chedule yearly eye and hearing chec)s
:eu)emia" 3eferral
#
3efer the patient to a hematology<oncology
specialist if &B&, differential, history, and
physical examination raise suspicions of
leu)emia
#
3eferral should be made in a timely manner to
avoid complications related to delays
#
Facilitate clear, concise, and current
communication with the hematology<oncology
specialist
:ymphoma" Incidence
# :ymphoma generally involves a painless, unilateral, enlarging, firm
mass
#
OE? of cases are located in the nec), often in the upper third
#
/on'$odg)in lymphoma is a malignant disease of the lymphoid
tissue in which no 3eed'(ternberg cells are present
# /on'$odg)in lymphoma affects nine of every 7EE,EEE people>
incidence pea)s between O and 77 years
# $odg)in lymphoma affects =.@ of every 7EE,EEE persons>
approximately O@EE cases each year in the 2nited (tates
#
$odg)in lymphoma affects men more often than women
# 4pproximately C=? of childhood cancers are 4::, brain tumors, and
lymphoma
# From birth to age C, lymphoma is one of the top four malignancies
# Between the ages of C and 78 years, $odg)in lymphoma is one of
the top two malignancies
#
In children older than age 78, $odg)in lymphoma is the most
fre1uent malignancy of the head and nec)
# 3is) is increased in people with autoimmune diseases or other
immunodeficiencies
:ymphoma" %athophysiology
#
.alignant transformation of an uncertain progenitor cell
leads to the development of the 3eed'(ternberg cell or
other malignant cell
#
Disease spreads to lymphoid tissue and eventually to
nonlymphoid tissue by way of the lymphatic drainage
pathwayX
#
Immature lymphocytes migrate through central lymphoid
tissues" the bone marrow *probable central lymphoid
tissue for B lymphocytes- and the thymus *central
lymphoid tissue for T'lymphocytes-
#
.ature lymphocytes later reside in the T' and B'
lymphocyteIrich areas of the peripheral lymphoid tissues
#
:ymphocytes from the mucosal'associated lymphoid
tissues circulate throughout the body in a separate
pattern from other lymphocytes
#
$odg)in disease spreads in an orderly, contiguous, and
predictable manner
:ymphoma" %athophysiology
Current complaint
#
%ainless, enlarged lymph nodes
#
Fever
#
/ight sweats
#
2nexplained weight loss of more than
7E? of the normal body weight
#
Fatigue
#
4norexia
:ymphoma" $istory
Medical history
#
3ecent infections that may have caused enlarged lymph nodes
#
&ongenital or ac1uired immune dysfunction
# Blood'product use
# $uman immunodeficiency virus *$ID- infection
# Medication history
#
&hronic treatment with phenytoin *increased ris) of lymphoma-
Family history
# :ymphoma
# Other malignancies
# Psychosocial history
#
6xposure to someone who is ill
# 6xposure to unusual or uncommon animals
# Ingestion of unpasteuri+ed mil)
# Foreign travel
# 3is) factors for $ID infection
:ymphoma" %hysical 6xamination
Examination
#
3ecord vital signs, including weight and, in
children, growth
#
4ssess the patient!s general appearance
#
&hec) common and uncommon node sites
*particularly the nec), axilla, supraclavicular
area, and groin- for enlarged nodes, noting si+e,
shape, consistency, location, fixation, and
duration and rate of change
#
&hec) for hepatomegaly and splenomegaly
:ymphoma" %hysical 6xamination
Diagnostic procedures
#
&B& with differential
#
&hemistry panel
#
(edimentation rate
#
:iver' and renal'function tests
#
&hest radiography
#
&omputed tomography or magnetic'resonance imaging
#
6xcisional lymph'node biopsy
#
:ymphangiography
#
6xploratory laparotomy, with splenectomy in some
patients
#
Bone'marrow biopsy
:ymphoma" %hysical 6xamination
Differential diagnosis
#
$odg)in lymphoma vs. other lymphomas
#
.ononucleosis *fatigue, malaise, and fever with
impressive cervical lymphadenopathy and
atypical lymphocytosis-
#
(arcoidosis
#
4c1uired immunodeficiency syndrome *4ID(-
#
4utoimmune diseases such as rheumatoid
arthritis and lupus *arthralgias, muscle
wea)ness, unusual rash-
#
&at'scratch fever *causes enlarged lymph
nodes-
:ymphoma" Treatment, 6ducation,
Follow'2p, and 3eferral
#reatment
#
(ubtotal or total nodal irradiation
#
&hemotherapeutic regimen
Education
# Discuss the effect of therapy on gonads and explain sperm ban)ing
# Inform patients of the ris) of secondary malignancies
Follo%-up
#
The need for and timing of follow'up is determined by the oncologist
&eferral
# 3efer patient to a surgeon for excisional biopsy
# The patient should be referred to an oncologist if biopsy reveals
malignancy
#
3efer the patient and his or her family to an oncology support group
such as the :eu)emia and :ymphoma (ociety
(olid Tumors" Incidence
#
In adults, most intracranial tumors are supratentorial
# 4lthough pediatric malignancy is rare compared with cancer in
adults, brain tumors are one of the top three childhood cancers
# Tumors contribute significantly to the morbidity and mortality of
childhood diseases
# In children, infratentorial lesions account for CE? of &/( tumors
#
Other common tumors in children between birth and B years of age
are neuroblastoma, retinoblastoma, and ilms tumor
# .usculos)eletal cancers present as a mass, a painful extremity or,
occasionally, a pathologic fracture
# 3is) is increased in individuals with hereditary cutaneous
syndromes *xeroderma pigmentosum-, neurofibromatosis, or
chromosomal abnormalities *e.g., Down syndrome-
#
4 history of cancer puts an individual at increased ris)
# Intrauterine agents *diethylstilbestrol YD6(Z- have been lin)ed to
solid tumors, as has radiation
# .etabolic diseases *e.g., alpha'7 antitrypsin deficiency- increase
ris)
(olid Tumors" %athophysiology
#
4 se1uence of cellular and tissue changes leads from
dysplasia to neoplasia in situ to invasive neoplasia
#
Distinguishing dysplasia from neoplasia in situ is difficult
#
:oss of normal tissue architecture signals the
development of neoplasia
#
(uch changes are most commonly seen in the
s1uamous epithelium of the uterine cervix, the epidermis
of sun'exposed s)in, and the colonic and gastric mucosa
after longstanding inflammation
#
4ltered cell turnover during inflammation probably allows
local environmental factors to cause genetic
abnormalities, leading to neoplasia
(olid Tumors" $istory
Current complaint
#
0ait problems and ataxia *consider infratentorial lesions-
#
$eadaches, vomiting, and lethargy or other signs of
increased intracranial pressure *consider supratentorial
lesion-
#
Dull aching pain in limbs, especially at night *consider
osteosarcoma-
#
Blood in stool
#
Fever
#
4norexia
#
&onstipation and difficulty voiding *consider spinal'cord
tumor-
#
4bdominal swelling
(olid Tumors" $istory
Medical history
#
6xposure to $T:D'I virus
#
3ecent viral illness
#
Illnesses and surgeries
#
4llergies
#
Immuni+ations and screenings
Medication history
#
6xposure to cytotoxic drugs or chloramphenicol
#
.edications, including home remedies, OT&
drugs, vitamin and mineral supplements, and
borrowed medicines
(olid Tumors" $istory
Family history
#
0enetic abnormalities
#
&ancer
Psychosocial history
#
3elationships with family and friends
#
Tobacco, alcohol, and drug use
#
Occupational exposure *e.g., radiation,
chemotherapeutic agents, ben+ene-
(olid Tumors" %hysical
6xamination
Examination
# 4ssess the patient!s general appearance
# 4ssess vital signs for hypertension *may indicate renal tumor- or fever
without source
# 4s) about unexplained weight loss
# :oo) for signs and symptoms of infection
# Be alert to signs and symptoms of increased intercranial pressure
# Inspect the s)in for excessive bruising, petechiae, pallor, rashes, and
infections
# &hec) for lymph'node enlargement
# 6xamine the eyes for fundi or papilledema
# :isten for murmurs resulting from anemia, whee+ing, and decreased breath
sounds caused by a possible mediastinal mass
# %alpate the abdomen for hepatosplenomegaly *always abnormal- or an
abdominal mass
# &hec) male patients for testicular enlargement
# Inspect the musculos)eletal system for painful masses, muscle atrophy, or
limp *osteosarcoma-
(olid Tumors" %hysical
6xamination
Diagnostic procedures
#
3adiography
#
&omputed tomography
#
.agnetic'resonance imaging
Differential diagnosis
#
Benign tumor
#
&ommon childhood cancers
(olid Tumors" Treatment
The treatment course for solid tumors
depends on the type of malignancy
#
3adiation
#
&hemotherapy
#
(urgery
#
Brachytherapy
#
$yperthermia
#
Biological therapies
#
&omplementary<alternative therapies
(olid Tumors" 6ducation
#
6xplain possible preventive measures
# 6xplain to the patient and family the diagnosis and treatment
plan
# Discuss the expected and toxic side effects of chemotherapy
# %rovide clear supportive'care guidelines to the family and
primary'care provider
#
6xplain medication and immuni+ation guidelines
# arn against using rectal thermometers, suppositories, or
tampons
# 3ecommend the use of a soft toothbrush or toothettes for
oral care and, if a central line is in place, antibiotic
prophylaxis before dental wor)
#
Tell the patient and family to call for a &B& with differential if
the patient becomes febrile to rule out fever resulting from
neutropenia
(olid Tumors" Follow'2p
#
3egular follow'up with specialists should be
ensured, especially for children who are cancer
survivors, starting 8 years after treatment is
complete
#
Weep a summary of cancer treatment
#
(chedule regular dental follow'up visits,
especially if treatment was administered before
@ years of age
#
(creen regularly for depression
#
(chedule yearly eye and hearing chec)s
(olid Tumors" 3eferral
#
3efer the patient to a hematology<oncology
specialist if &B&, differential, and history and
physical'examination findings indicate the
possibility of leu)emia
#
3eferral should be made in a timely manner to
avoid complications related to delays
#
Facilitate clear, concise, and current
communication with the hematology<oncology
specialist