PLATELET DISORDERS AND

BLOOD TRANSFUSION

Tuesday, December 18, 2018 1

.
I.PLATELETS DISORDERS
 1.HEMOPHILIA
Hemophilia is an X-linked recessive hemorrhagic disease.
It is a genetic disease due to mutations in the F8 gene
(hemophilia A or classic hemophilia) or F9 gene (hemophilia
B or Christmas disease).
Patients with hemophilia have normal bleeding times and
platelet counts.
TYPES OF HEMOPHILIA
 Hemophilia A, which is caused by a lack of clotting factor
VIII. Approximately 85 percent of hemophiliacs have type
A disease.

 Hemophilia B, which is caused by a deficiency of factor

IX.
cont
6
 PATHOPHYSIOLOGY

 The patient is missing a coagulation factor(FVXXX and FIX) that is

essential for normal blood clotting and as a result the blood does not
clot when the patient bleeds.
 It is an X-linked recessive inherited disorder, passed on so that it
presents symptoms in males, and rarely in females.
 FVIII deficiency arise from a large inversion that disrupts the FVIII
gene. Deletions, insertions, and point mutations account for the
remaining 50-60% of the F8C defects that cause hemophilia A.
 Low FVIII levels may arise from defects outside the FVIII gene, as in
type IIN von Willebrand disease, in which the molecular defect resides
in the FVIII-binding domain of von Willebrand factor.
 ETIOLOGY
Hemophilia types A and B are inherited diseases passed on to
children from a gene located on the X chromosome. Females
have two X chromosomes, and males have one X and one Y
chromosome. A female carrier of hemophilia has the
hemophilia gene on one of her X chromosomes.
When a female hemophilia carrier is pregnant, there is a 50/50
chance that the hemophilia gene will be passed on. If the gene
is passed on to a son, he will have the disease. If the father has
hemophilia but the mother does not carry the hemophilia
gene, then none of the sons will have hemophilia disease, but
all of the daughters will be carriers.
 Carriers of the hemophilia gene usually have normal
levels of clotting factors but may bruise easily, bleed more
with surgeries and dental work, and/or have frequent
nosebleeds or excessive menstrual bleeding.
 SIGNS AND SYMPTOMS
 Tender joints due to bleeding
 Swelling of knees, ankles, hips, and
elbows due to bleeding
 Blood in stool (tarry stool) due to GI
blood loss
 Blood in the urine (hematuria)
 THE PARACLINICAL EXAMINATIONS AND RESULTS

 PTT (Partial Thromboplastin time) prolonged.

 PT normal.
 Fibrinogen level normal.
 Decrease in clotting factor VIII found in blood serum in
Hemophilia A.
 Decrease in clotting factor IX found in blood serum in
Hemophilia B.
 Clotting factor levels testing
 Complete blood count (CBC)
 Assessment of bleeding times
 Genetic testing
Cont’
 Diagnosis of affected individual and carries can be done
before birth
Through chorionic villus sampling or amniocentesis. Genes
for clotting VIII and IX are located near the terminal long
arm of the X chromosome.
 Diagnosis can also be based on the history taking ,and
physical assessment.
TREATMENT

The goal of medical management is to control the bleeding by

replacing the missing clotting factor. replacement therapy is
indicated for when the child experiences mild or major
hemorrhage or face life threatening situations.
Treatment depends on the type and severity of the hemophilia.
The goal of hemophilia treatment is preventing bleeding
complications (primarily head and joint bleeds),
 For hemophilia A administer factor VIII concentrates.
 Cryoprecipitate.
 DDAVP (desmopressin) for patients with mild deficiency.
 NURSING MANAGEMENT
NURSING DIAGNOSIS:
 Acute pain
 Impaired gas exchange
NURSNG INTERVENTIONS:
 No IM injections.
 No aspirin.
 To stop bleeding:
 Elevate site.
 Apply direct pressure to the site.
 Explain to the patient:
 Wear a medical alert identification.
 Contact physician for any injury.
 Avoid situations where injury might occur.
COMPLICATIONS

 Deep internal bleeding

 Damage to joints and cartilage
 Infection.
 Adverse reaction to clotting factor treatment
Cont’
Damage to joints and cartilage
 Left untreated, frequent internal bleeding may cause arthritis
 Infection. People with hemophilia are likelier to have blood
transfusions, increasing their risk of receiving contaminated
blood products
 subchondral bone-cyst formation
 permanent deformities and misalignment
 loss of mobility
 extremities of unequal lengths
ADVERSE REACTION TO CLOTTING
FACTOR TREATMENT

In some people with hemophilia, the immune system has

a negative reaction to the clotting factors used to treat
bleeding. When this happens, the immune system
develops proteins (known as inhibitors) that inactivate the
clotting factors, making treatment less effective.
 PREVENTIVE MEASURES
parents can prevent bleeding complications by:
 Purchasing soft toys with rounded corners for young
children.
 Dressing children in padded clothing and helmets when
they are learning to walk or becoming more active.
 Evaluating contact sports in school for risks of injury to the
child.
 Having properdental hygiene.
 Not giving the child aspirin and aspirin-containing
products if recommended by the child’s physician. These
products have been linked to bleeding problems
CONT’
 Health care providers may take these preventative steps:
 Give immunizations under the skin (subcutaneous) instead of in the
intramuscular) to prevent deep muscle bleeds.
 Recommend surgery for joint hemorrhages and/or immobilization.
Rehabilitation of the affected joint may include physical therapy and
exercise to strengthen the muscles around the area.
 Recommend infusions of replacement blood factors to increase the
child’s clotting levels before surgery or dental work.
 The child also may receive infusions during and after the surgery to
maintain the clotting factor levels and to improve healing and
prevention of bleeding after the procedure.
 Blood transfusions may be necessary if significant blood loss has
occurred.
Cont’
 REFERENCES: hand book integrated management of
child hood illness, World Health Organization,
Department of Child and Adolescent Health and
Development (CAH), Avenue Appia 20, CH-1211 Geneva
27,Switzerland,Page 47.
 Pediatric nursing,caring of children,3rd edition,jane
w.ball,ruth c.blinder chapter15 alterationin hematologic
function,platelet disorders page516.
2.THROMBOCYTOPENIA
Definitions

 Thrombocytopenia is defined as as decrease in the

number of circulating platelets. Normal platelets counts
ranges 150000 to 450000/mm3 . Platelets are irregular,
disc- shaped element in the blood that assists in blood
clotting. It includes :
 Thrombocytopenia due to increased destruction
(Immune (idiopathic) Thrombocytopenic
Purpura,
 Iso immune thrombocytopenia in the neonate)
 CONT’

 Thrombocytopenia due to decreased platelet

production
 Thrombocytopenia related to peripheral
consumption (Disseminated intravascular
coagulation, and Thrombotic thrombocytopenic
purpura(TTP)
 Thrombocytopenia due to hypersplenism (Normally
spleen have one to third of platelets of the body in
Hyperspleenis, 90% of platelets I the blood are
trapped(collected) in enlarged spleen)
 CONT’
 Heparin- induced thrombocytopenia(HTP);
Occurring 8-10 days after reexposure to heparin.
 Thrombocytopenia of neonatal sepsis and
congenital infection: This occurring is related to
overwhelming bone marrow suppression or to peripheral
consumption. Congenital infections with cytomegalovirus,
Rubella, toxoplasma may cause Impaired marrow
production or peripheral consumption related to
splenomegaly.
Etiology and pathophysiology

 Platelets are fragments of megakaryocyte produced in bone marrow

under the influence of thrombopoetin made by liver and kidney.
 The platelets enter the blood circulation and have life span of 7-10 days.
They cleared from the body by the spleen, liver and bonne marrow.

 Thrombocytopenia can arise due to decreased production of platelets

in the bone marrow (Primary malignancy like leukemia, viral infection,
cytotoxic drugs, radiation, bone marrow failure) , increased breakdown of
platelets in the blood steam, spleen or liver(ITP and DIC), hypersplenism
(due to infection, inflammation).

 Most common reason for thrombocytopenia in children is Idiopathic

Thrombocytopenic Purpura. Also called Immune system
thrombocytopenia.
 CONT’

 ITP occur when the immune system mistakenly attacks and

destroys platelets
 Acute, severe, or prolonged decreases from the normal
range can result in abnormal hemostasis that manifests as
prolonged bleeding from minor trauma or spontaneous
bleeding without injury.
 SIGNS AND SYMPTOMS

 Easy bruising of the skin, Petechiae, Bleeding in gastrointestinal

tract (including the mouth) and pulmonary system (eg,
hemoptysis),
 Blood in urine, black tarry stool and Menorrhagia.
 Bleeding may occur in vital organs, such as the brain, which may
prove fatal.
 In the acute type, onset may be sudden and without warning,
causing easy bruising, nosebleeds, and bleeding gums
 Aplastic anemia presents with bleeding.
 Paraclinical examination

 CBC to diagnose thrombocytopenia, RBC indice

reticulocytes to see if there is no Anemia that result from
Bleeding.
 Peripheral blood smear to rule out ITP, DIC, and TTP
and RBCS Fragmentation.
 If Malignancy suspected bone Marrow biopsy is done.
Treatment of thrombocythopenia
It depends on the cause of your child’s
thrombocythopenia and in some cases, treatment
maight not be necessary.
Treatment of thrombocythopenia may include
Corticosteroids or immune suppressing drugs,
Bone marrow transplantation for these
congenitals and blood platelet transfusion.
 Complication of thrombocytopenia

Severe Bleeding may leads to severe

anemia, confusion (neurological
changes) and kidney failure.
 Nursing intervention to the patient
with thrombocytopenia
 Use a soft toothbrush or gauze to clean the teeth.
 Avoid flossing.
 Avoid invasive procedures as much as possible, including
enemas, douches, suppositories, and rectal temperatures.
 Avoid intramuscular injections.
 To avoid injury when checking blood pressure, pump cuff
up only until pulse is obliterated.
 Avoid blood draws whenever possible. Use established
access sites or group draws into once daily
cont’
 Maintain pressure on intravenous (IV), blood draw, and other
puncture sites for 5 minutes.
 Encourage use of shoes or slippers when out of bed.
 Keep area clutter free to prevent bumps and bruises.
 Avoid use of drugs that interfere with platelet function, such as
aspirin products and nonsteroidal anti-inflammatory drugs.
 Administer stool softeners as ordered to prevent straining to have a
bowel movement.
 Move and turn patient gently to avoid bruising.
 Instruct patient to avoid blowing the nose.
3.IDIOPATHIC
(IMMUNE)THROMBOCYTOPENIC
PURPURA (ITP)
Definition
 is an acquired hemorrhagic disorder that is characterized by
an increased destruction of platelets because of antiplatelet
antibodies.
 The antibodies attach to the platelets, reduce their life span,
and lead to a platelet count below 100,000 mm3 but
occasionally as low as 5000 mm3.
 There are two forms of ITP: acute and chronic.
 The acute form, which occurs predominately in children,
often appears 1 to 6 weeks after a viral illness.
 Chronic ITP generally has no underlying viral association
and is often linked to immunologic disorders, such as lupus
erythematosus, or to drug reactions.
 Etiology and pathophysiology
 Although the precise cause remains unknown,
viral infections sometimes precede ITP in
children ( rubella or chickenpox)
 Occasionally medications such as sulfa drugs can
induce ITP.
 Acute ITP may occur after live vaccine
immunisations
 It is also associated with HIV
 Cont’
Anti-platelet autoantibodies that bind to the
patient’s platelets are found in the blood of patients
with ITP.
When the platelets are bound by the antibodies, the
RES or tissue macrophage system ingests the
platelets and destroying them hence decrease in
number.
The body attempts to compensate for this
destruction by increasing platelet production within
the marrow.
SIGNS AND SYMPTOMS

Common physical manifestations are:

 Easy bruising,
 Heavy menses,
 Petechiae on the extremities or trunk.
 Bleeding in gastrointestinal tract (including the mouth) and pulmonary
system (eg, hemoptysis), which is termed “wet purpura.”.
 Splenomegaly may present in children with acute ITP following viral illness
within a week.
 Patients may have an isolated decrease in platelets (less than 20,000/mm3
is common), but they may also have an increase in megakaryocytes
(platelet precursors) within the marrow, as detected on bone marrow
aspirate
PARACLINICAL EXAMINATIONS AND RESULTS
INTERPRETATIONS
 Complete blood count(CBC)
 Bone marrow aspirate.
 Viral titres, eg Epstein-barr Virus, rubella if a child is under 1 year.
 Immunoglobulins IgG, IgA.

Patients may have an isolated decrease in platelets (less than 20,000/mm3 is

common), but they may also have an increase in megakaryocytes (platelet
precursors) within the marrow, as detected on bone marrow aspirate.
The rest of CBC is normal, unless anemia is present after a history of
significant bleeding.
Sometimes, antibodies are detected in blood.
Treatment and prevention

The primary goal of treatment is a safe platelet count.

Because the risk of bleeding typically does not increase
until the platelet count is lower than 10,000/mm3, patients
whose counts exceed 30,000 to 50,000/mm3 may be
carefully observed without additional intervention
Pharmacologic option:
 CONT’

If the patient fails to respond within 1 to 4 months

or needs a high steroid dosage, splenectomy is
usually considered.
Splenectomy is effective because the spleen is a
major site of antibody production and platelet
destruction; research suggests that splenectomy is
successful 85% of the time
NURSING MANAGEMENT

Interventions:
Nursing care focuses on controlling and reducing the number of
bleeding episodes;
 Teach a parent to use acetaminophen rather than aspirin to control
pain.
 Prevent children to fall dawn.
 Manage children’s environment
 Provide safety
 Health education
 Frequent Surveillances
 Medication management
4. DISSEMINATED INTRAVASCULAR
COAGULATION (DIC)
Definition
DIC is a serious bleeding and thrombotic disorder
that results from abnormally initiated and
accelerated clotting. Subsequent decreases in
clotting factors and platelets resulting in
widespread clot formation in small vessels
throughout The body. Which may lead to
uncontrollable hemorrhage.
 Etiology
DIC can occur in the following conditions:
Solid tumors and blood cancers (particularly acute promyelocytic leukemia)
Obstetric complications: abruption placentae, pre-eclampsia or eclampsia,
amniotic fluid embolism, retained intrauterine fetal demise, septic abortion,
post-partum hemorrhage
Massive tissue injury: severe trauma, burns, hyperthermia, rhabdomyolysis,
extensive surgery
Sepsis or severe infection of any kind ,bacterial (Gram-negative and Gram-
positive sepsis), viral, fungal, or protozoan infections
Transfusion reactions (i.e., ABO incompatibility hemolytic reactions)
Severe allergic or toxic reactions (i.e. snake venom)
Large aortic aneurysms
Pathophysiology

The pathophysiology involves an over activation of the clotting mechanisms

with both enhanced fibrin production leading to small clots and fibrinolysis
leading to enhanced bleeding.
As its name implies, tiny clots accumulate in the microcirculation (capillaries)
throughout the body, depleting the blood supply of its clotting factors.
These microemboli interfere with blood flow and lead to ischemia and organ
damage.
As the clots begin to lyse, fibrin degradation products (FDPs) (which have an
anticoagulant property of their own) are released.
The FDPs, along with decreased levels of clotting factors in the bloodstream,
lead to massive bleeding internally from the brain, kidneys, adrenals, heart, and
other organs, or from any wounds and old puncture sites
SIGNS AND SYMPTOMS OF DIC

Petechiae as clotting factors are lost

Purpura as clotting factors are lost
Severe hemorrhage as clotting factors are lost
Uncontrolled postpartum bleeding
Tissue hypoxia from micro emboli
Hemolytic anemia, as cells are destroyed trying to pass through partially
blocked vessels chest, shortness of breath, leg pain, problems speaking,
or problems moving parts of the body.
Unexpected bleeding, oozing from puncture sites (venipuncture, IVs,
surgical wounds)
DIAGNOSTIC MEASURES AND
RESULTS
Potential complications

 Renal failure
 Gangrene
 Pulmonary embolism or hemorrhage
 Altered level of consciousness
 Acute respiratory distress syndrome
 Stroke
TREATMENT and PREVENTIONS
Treatment needs to decrease coagulation ability and replace clotting
components (to prevent further bleeding)
Transfusion:
Packed RBC to replace what has been lost due to bleeding.
Fresh frozen plasma replaces coagulation factor deficiency.
Platelets replaces needed cells.
Cryoprecipitate replaces fibrinogen.
Administer anticoagulant drugs to decrease coagulation; not done in
all patients:
heparin
Bed rest
CONT’
Primary Prevention
Primary prevention is early treatment of conditions or
disorders known to precipitate DIC.
Both early identification and prompt treatment of those
conditions or disorders are required to reduce the chance
of developing DIC.
Secondary Prevention
The most effective prevention for DIC is to actively and
aggressively treat the underlying disorder.
Nursing management
Nursing care focuses on:
 Monitor for bleeding from obvious sites (wounds, suture lines,
venipuncture, etc.) and occult sites (GI, urine). Explain to the patient:
 Avoid situations that might cause bleeding
 Assessing the bleeding, preventing further injury, and administering
prescribed therapies, observe for petechial, ecchymosis, and oozing
every 1 to 2hours.
 Examine stool for presence of blood and measure blood loss.
 Then measure intake and output. Because all body system can be
involved carefully assessment of all body systems is needed on
continual basis.
 Monitor prescribed therapy (transfusion, anticoagulant therapy) and
report any signs of complications
II.BLOOD TRANSFUSIONS
INTRODUCTION
Transfusion is a therapeutics based on the use of human
blood and its derivatives.
Without transfusion Medicine, a lot of deaths would be
registered in the Maternities because of obstetrical
hemorrhages, in Pediatrics because of anemia from
malaria and other parasites and in Surgery because of
accidents. The transfer of blood or blood components
from one person (the donor) into the bloodstream of
another person (the recipient).
INDICATION OF BLOOD TRANSIFUSION
 Improve oxygen carrying capacity to maintain adequate
hemoglobin and relieve symptomatic anemia
 Maintain adequate pre-operative hemoglobin>8g/dl
 Chronic transfusion, every 2-4 weeks for different
complications in certain diseases such sickle cell anemia, beta-
thalassemia major, severe hereditary spherocytosis,
myelodysplasia
 CONT’
Platelet transfusion with thrombocytopenia:
Platelet count 5-10000/with failure of platelet production
Platelet count< 30000/ɥl in neonates with failure of platelet production.
Platelet count<50000/ɥl in stable premature infant with, active bleeding,
before intensive procedure with failure of platelet production
Platelet count < 100000/ɥl in sick premature infant with active bleeding,
and before an invasive procedure in patient with DIC
Without thrombocytopenia:
Active bleeding with qualitative platelet defect
Unexplained excessive bleeding in patient undergoing cardiopulmonary
bypass.
Transfusion Procedure in pediatry
Typical transfusion is 10ml/kg given over 3 to 4 hours.
May need second transfusion (preferably from same donor) if anemia not adequately
corrected.

Volume of transfusion

• To calculate volume based on observed and desired hematocrit, estimated blood

volume of 80 ml/kg
Calculation: (desired hematocrit – observed hematocrit) x weight x 80 ml
Hematocrit of blood to be given (typically 60-90%)

NB. Whole blood should be given to correct the anemia of rapid blood loss.
If hematocrit is not available: give 10ml/kg and monitor
TYPES OF BLOOD TRANSFUSIONS
Blood is transfused either as whole blood (with all its parts) or,
more often, as individual parts namely:
Red Blood Cell Transfusions
Platelets and Clotting Factor
Plasma Transfusions
BLOOD MATCHING

There are 4 main blood groups: A, B, AB and O

This means that: Group A Persons have antigen A on their RBC, Group
B: Persons have B antigens on their RBC
Group AB Persons have both A&B antigens on their RBC and Group
O Persons not having antigens (A, B)
CROSS-MATCHING OF BLOOD
Cross-matching of blood is carried out to determine the compatibility.
The serum of the patient is reacted in tube against the red cells of the
patient of the donor and the presence or absence of agglutination is
noted prior to the cross-matching
Group O Gives Blood to group O, A, B, AB, but he receives blood from
only the group O
Group A gives blood to group A, AB but he receives blood from Group
A, O
Group B gives blood to group B, AB but receives blood from O, B
Group AB, gives blood to group AB, but receives blood from group O, A,
B and AB.
THE RHESUS SYSTEM

There are 6 antigens involved in rhesus blood groups the antigen most
frequently involved in incompatibility and hoemolysis is anti D.
This antigen is present in 85% of the population.These people are known
rhesus positife(Rh +) .
This antigen is also absent in 15% of the population.These one are known as
rhesus negative(Rh-) .The problems occur when a person rh- is given rh+
blood: on first occasion ,it will cause no harm to the receiver but he will
produce antibodies against antigen D and further transfusion with Rh+ Blood
will produce hemolysis.
Another example is that a rh- woman when is married to a rh+ man: Their
first child will be born and live without any problem, but the following
pregnancies will end up ,with obortion ,prematurity and children if they are
born may not live
 POTENTIAL COMPLICATIONS OF
BLOOD TRANSFUSION
 Acute hemolytic accident Caused by: A, B, O or rhesus
incomptatibility improperly stored blood,proper cross-matching and
intradonor incompatibility.
 Bacterial, viral contamination
 Delayed hemolysis
 Allergic Reaction
 Bleeding Tendencies
 Circulatory overload
 Hemosterosis
 Hypothermia
 Hypocalcemia