.......................................................................

.............





:


: : : :



3
RBCs
WBCs
platelets
2/3 hematology
, bleeding disorder 1/3

: points 4
Definition
)causes(etiology
C/P
Investigations
what is the definition of anemia



Pallor
Pallor
1


.......................................................................
.............
anemia


Pallor
Pallor

Pallor
Pallor Anemia
pallor

anemic pale
pallor anemic


CBC



......

defintion of anemia
11 % dl /


gram /dl 11
definition

11 Per dl
anemic male or female
regardless age or sex

pallor


pallor anemia
2


.......................................................................
.............



complete blood count CBC
gram / dl 11

target
extermities


18

neonatal period
Infant period
childhood period 12 10
adlocent
adult
target
Phsiology


hematology

fetus Intra utrine
placenta
placenta good source for oxygen supply
lung

oxygen supply
Lung


.......................................................................
.............


fetus
fetal hemoglobin
Hb F

Hb F
oxygen dissociation


fetus


net result
Intra utrine cells fetus
Partial hypoxia

kidney has oxygen sesors

PO2
hypoxia
erythropiotein
Liver
erythropiotein hypoxia
stimulation of RBCs synthesis
Liver spleen
RBCs

hemoglobin
gram / dl 18-22

Polycythemia
) blood


4


.......................................................................
.............
18 ........
... stimulus ...
hypoxia
18 ....
............ 9


....
9
Normal
adult -14
15 Puberity 15 15


72 15

N.B. reduction of haemoglobin in old age is adisease

15

18 9 9 ...... 15

Lung
hypoxia
erythropiotein stimulation of RBCs synthesis
hemoglobin
... hemoglobin gram / dl 9


.......................................................................
.............


physiological anemia of the neoborn

MCQ
phsiologyical anemia of the neoborn

hemolysis of the RBCs (a
Nutritional anemia (b
bleeding tendency (c
(d erythropiotein level
D erythropiotein level
weeks 6 Hemoglobin
erythropiotein
stimulation of the RBCs

Etiology of anemia

RBCs count
) (

synthesis
excess loss

excess loss of RBCs

hemorrhagic anemia (a active bleeding
Hemolysis of the RBCs (b


.......................................................................
.............
synthesis
Decrease synthesis of the RBCs

(a bone marrow
bone marrow failure
requirement of the bone marrow (b

RBCs

Hemoglobin cytoplasm cell membrane

:
cell membrane vitamin E
cell membrane
:
hemoglobin
heme + globin
globin
protein cytoplasm cell membrane
Heme
iron + protoporphrin
iron carry
Protoporphrin cupper Vit. B6

stem cells
Mother cells
platelets WBCs RBCs
Vit. E
anti oxidant
synthesis


.......................................................................
.............
RBCs free radicals
RBCs Hemolysis


enumerate causes of nutritional anemia and discuss how to
diagnose one of them

Nutritional anemia
nutritional anemia
Iron deficiency
cupper deficiency
vit. B6 deficiency
protien deficiency
Vit. E deficiency
Vit. D deficiency
vit. B12 and folic acid deficiency

How to diagnose one of them
the most common cause
Most common type of nutritional anemia

Iron deficiency anemia

Nutritional anemia
iron deficiency anemia

title nutritional anemia


.......................................................................
.............



Nutritional anemia
anemia
nutritional anemia
anemia

exception
Pancytopenia
pancytopenia
pancytopenia
RBCs
RBCs WBCs Plateles

stem cells

stem cells
RBCs WBCs Platelets

nutritional anemia
anemia
Vit B1 Vit B12 folic acid
pan cytopenia



bone marrow failure
bone marrow failure
bone marrow failure
bone marrow
stem cells
un differniated cells
stem cells un differentiated cells


.......................................................................
.............
Un differeniated cells
Organ
stem cell
RBCs
WBCs platelets

receptors
stem cells
receptors erythropiotein hormone
erythropiotein hormone receptor
erythropiotein hormone receptor
erythropiotien
stem cells
RBCs
RBCs
erythropioesis

receptor
thrombopiotein

erythropiotein
thrombopiotein
thrombopiotein receptor
stem cells
Platelets
Megakaryocytes
Mother cells of the platelets
plateletes

receptor
inflammatory cytokines
10


.......................................................................
.............
interlukin tumor necrosis factor

receptor
Interlukin
cytokines
receptor stem cells
stem cells
receptor
Inflammatory process

interlukin

cytokines
Inflammatory process
WBCs

iron deficiency anemia
daily requirement of iron mg/kg /day 3-2
sources of iron
animal plant
Plant very complex
simple formula

iron in animal protien

ferric iron
stomach

ferric ferrous
acidic media
.N.B
acidity iron absorption
11


.......................................................................
.............
breast feeding
deficient in iron
acidic alkaline
absorption
artificial milk
deficient in iron
alkaline
absorption
iron
breast feeding
iron PH stomach

antacid
... ... gastric erosion antacids
ulcers stomach

alkaline iron absorption

Iron
iron ferric ferrous


ferrous iron upper part of the duodenum
2nd and 3rd part of the duodenum
PH alkaline
absorption
mucosa of the duodenum
carrier protien apoferritin
iron
iron ferritin
carrier protien blood
blood transferrin
stores

12


.......................................................................
.............
Liver
6

???? why iron has carrier protein in the blood

Iron free in the blood
deposition inside the tissues
hemosidrosis

| |
apoferritin Iron Lumen of the duodenum
lumen duodenum

stores enough 6

stores

7th, 8th, and 9th months

stores

:

1/3
: gram 2500

stores
intra utrine growth retardation
.N.B
iron deficiency anemia
breast feeding

13


.......................................................................
.............
teeth
breast milk iron
suppose 2 mg /kg /day

breast feeding 1mg /kg /day

iron breast feeding absorption
stores
2mg /kg /day

Stores 6
6 iron deficiency anemia

iron

"

"

causes of iron deficiency anemia

decrease intake
delayed weaning
breast feeding only
absorption of iron
absorption of iron
a- alkaline media in the stomach and duodenum
b- other content of food
Positive or negative
absorption of iron
Positive absorption of iron
vitamin C acidity
protiens amino acids
14


.......................................................................
.............
negative iron absorption

chemical reaction with iron
decrease absorption of iron
...
phytate and oxalate
oxalate
phytate catch iron
absorption of iron


oxalate
oxalate iron absorption of iron
oxalate

decrease the stores of iron

excess requirement of iron
adolescent
rate of growth
5 requirements

iron deficiency anemia

excess loss of iron
Iron RBCs
RBCs
chronic blood loss
RBCs iron
iron deficiency anemia
.N.B

(:

15


.......................................................................
.............
some infants
sensitive to cow milk
something like dysentry
diarrhea, mucous and blood
clinical manifestations of iron deficiency anemia
:Symptoms of anemia*
FAHM general symptoms
headache, dizziness and syncopal attack
easy chest pain Palpitations chest pain
fatigability
intermittent claudication
specific symptoms *
anorexia
feeding centre brain

centres
cytochrome system
main component of cytochrome system
iron like RBCs (HB)
iron
feeding centre signals
.N.B
problem
):


signals cytochrome system


Pica

16


.......................................................................
.............

true pica and pseudo pica
pseudo pica
true pica
sever iron deficiency anemia

Signs of iron deficiency anemia

general anemia, pallor and tachycardia
specific

atrophy of the tongue
Nails
atrophy of the nail bed
spooning of the nails
as caused by atrophy of the nail bed
ischemia sever anemia
abdomen
splenomegaly % 15-10

investigations
CBC
micro cytic hypochromic anemia
hemoglobin gram /dl 11
) MCV ( MCV
mean corpscular volume

femtoliter 60 Microcytic

MCH picogram 26

iron deficiency anemia synthesis
zero
17


.......................................................................
.............
blood film
no abnormal cells
serum ferritin of serum iron

iron binding capacity
free
transferrin 3 iron
normal iron
transferrin
...


iron deficiency anemia
serum iron
iron binding capacity


serum iron serum ferritin

serum iron serum ferritin

serum ferritin acute phase reactant
acute phase reactant
iron deficiency anemia
acute follicular tonsillitis
CRP ... serum ferritin
iron deficiency anemia Infection serum
ferritin serum ferritin Normal high iron
deficiency anemia
serum ferritin Infection
iron
serum iron serum ferritin
iron ferritin
... iron component Iron
ferritin protien carrier iron
18


.......................................................................
.............
serum ferritin protein Kwashiorkor
iron deficiency anemia
serum ferritin
Pan hypoprotienemia
serum ferritin serum iron
iron binding capacity
iron binding capacity serum iron
iron binding capacity
.......

heme globin
heme Iron protoporphrin
globin

diagnostic iron deficiency anemia

free erythrocyte protoporphrin

megaloblastic anemia
notes
Megaloblastic anemia

defect in RBCs synthesis due to B12 and folic acid
etiology
Vit. B12 and folic acid
Vit. B12 and folic acid deficiency
sources of Vit. B12
animal only

B12 micro gram per day 1
.N.B

19


.......................................................................
.............
in neonates .... stores in liver
15 full term

stomach
parietal cells
carrier protien ) ( Intrinsic factor
B12 Intestine
terminal ileum
receptors
receptors Intrinsic factor

B12 intrinsic factor circulation
Intrinsic factor
B12
Intrinsic factor


stool

B12 deficiency

intake sever malnutrition
Problem in the stomach

gasterectomy partial or total
intrinsic factor
B12
stores B12
deficiency in intrinsic factor
Intrinsic factor
B12 megaloblastic
pernicious anemia

megalobalstic anemia pernicious anemia

20


.......................................................................
.............
deficiency of intrinsic factor perinicious anemia
perinicious anemia
megaloblastic anemia
perinicious anemia megaloblastic anemia

causes of perinicious anemia

congenital defect in parietal cells
Intrinisic Praietal cells
factor
jevuenile type of perinicious anemia
autoimmune
parietal cells antibody
common in adult
adult type of perinicious anemia
problem in pathway through intestine
consumption of B12 and intrinsic factor
Over growth of the bacteria

diphlopotherium latem
blind loop
problem in terminal ileum
site of absorption
surgically resection
granulomatous disease
inflammatory bowel disease
Stores
Liver
Folic acid

plant animal

gram / day 1 daily requirement

liver absorption

21


.......................................................................
.............
liver can store folic acid

active form of folic acid is folinic acid
folic acid reductase enzyme

causes of deficiency of folic acid

Intake
folic acid
goat milk

decrease absorption
absorption
malabsorption syndrome
excess consumption of the stores

bone marrow

8 bone marrow hemolytic anemia

stores
block of the reductase
block folic acid
methotrexate
MCQ
patient take methotrexate after 2 weeks manifestations of anemia +
petichea + purpura and there is history of repeated infection the
: main line of treatment is
packed RBCs (a
folic acid (b
B12 (c
folinic (d
folinic acid folic acid d
active form
reductase block methotrexate
22


.......................................................................
.............
folic acid folinic
folinic

mitosis brain cell germ cell

clinical presentation
Mitosis
Mitosis total number of cells
parallel
mitosis


promotor gene
Organ
order

cytoplasm nucleus
cytoplasm transcript
Nucleus polymerase
polymerase active
methyl group
Methyl group
folic acid
methyl group
methyl group folic acid
B12
.N.B
folic acid methyl group
B12 methyl group folic acid
Pathway
Mitosis
DNA transcriptase
RNA
23


.......................................................................
.............
duplication of cyoplasm
cytoplasm
DNA

neucleus

total number
.N.B
megaloblastic anemia

megalo
blast malignant
)
Hematology
(:
blast
blast
blast
notes (

mitosis

hematological cells
RBCs days 120
platelets 400 days 21-7
WBCs 4000 11.000 days 15-7
normal bone marrow
% 2-1 reticulocytes

clinical pictures
pan cytopenia
degeneration of Schwann cells
brain cells mitosis

24


.......................................................................
.............
glossitis
failure to thrive
stomatitis

Notes Investigations

megaloblast


CBC
pancytopenia
synthesis
MCV
femtoliter 100
macrocytic anemia
MCH MCHC normal


(:
total number of RBCs

blood film
WBCs
hyper segmented
mitosis

platelets
immature
to be sure of your diagonsis
bone marrow

Mitosis
hypocellular
megaloblastic

25


.......................................................................
.............
causes
folic and B12
serum B12 and folic
neurological

B12 and folic acid

folic acid or B12
cherring test
figlu test .... folic test
!!!!!
Historical test
auto immune disorder

anti praietal cells antibodies
other type of perinicious anemia
suppressive therapy
B12 congenital
B12 parentral

folic acid deficiency
folic acid
B12 deficiency
mg B12 250
B12 B12
methyl folic acid


3
!!!!!!!!

B12 folic acid consumption

B12
folic acid

26


.......................................................................
.............




iron deficiency anemia
hemolytic anemia

enumerate causes of microcytic hypochromic anemia and discuss
how to diagnose one of them
anemia

patient 3years manifestions

anemia CBC
MCV 50
MCH 20
microcytic hypochromic anemia
enumeration

iron deficiency anemia (a
thalassaemia (b
lead pisoning (c
sidroblastic anemia (d
diagnosis iron deficiency anemia
diagnosis
clinical pictures Investigations
differential diagnosis

blood



27


.......................................................................
.............


diagnosis
diagnosis ....
clinical pictures Investigations
clinical diagnosis laboratory diagnosis

..... management
management treatment
........
Management
clinical picture investigations treatment
management clinical pictures investigation
treatment
diagnosis treatment
Undergraduates

disturbance mood

100



:
treatment !!!!


diagnosis treatment
diagnosis management ....
definition etiology ...... Pathogenesis

management diagnosis
definition Pathogenesis etiology
28


.......................................................................
.............


discuss

iron deficiency anemia
patient CBC
microcytic hypochromic anemia

blood film
no abnormal cells
serum iron

iron binding capacity

iron deficiency anemia

thalassaemia



hemolysis
Loss of the blood
hyperactive bone marrow
blood film thalassaemia
RBCs


anisocytosis
Hemoglobin electrophoresis

Hb F

29


.......................................................................
.............
lead pisoning

RBCs
lead deposit
RBCs

basophilic stibling of RBCs

sidroblastic anemia

cupper and B6 protoporphrin
sidroblastic anemia

Sidroblastic anaemia

sidroblastic anaemia MCQ
... bone marrow
normoblast
Normoblast reticulocyte mature RBCs
Normoblast normoblast iron
.... protopophyrin
protoporphyrin Iron haem ...
defect
protoporphyrin
iron normoblast bone marrow protoporphyrin

sidroblast
iron granules normoblast
protoporphyrin
sidrosis
normoblast sidroblast
sidroblast normoblast
protoporphyrin
(

30


.......................................................................
.............

treatment of iron deficiency anemia
prevention of iron deficiency anemia

any pregnant female
iron therapy 3
stores

screening for hypothyrodism
breast feeding
proper weaning


preterm
Iron supplmentation

| |
iron supplemention
full term

iron deficiency anemia

treatment of the underlying etiology
...
chronic blood loss
artificial milk
..
delayed weaning

.

iron therapy
Oral Parental
31


.......................................................................
.............
parental


Hyperpigmentation
coma
Parental
sever gastritis
Iron

iron parental
gastritis oral iron
dose
dose
elemental iron therapy
mg/ kg/ day 6
elemental iron
acutally absorbed

dose
3
3

cm / 3times per day 4


time


absorption iron
between meals


72
reticulocytic count


32


.......................................................................
.............
3 %5
bone marrow


hemoglobin


6-4
stores
6-4

| |

common side effect

stool

stool
iron absroption
Hyper pigmentation






packed RBCs
patient with iron deficiency anemia packed RBCs
gram / dl 6


anemic heart failure

33


.......................................................................
.............
Packed RBCs
hemoglobin gram /dl 10
iron therapy

gram/ dl 5

anemic heart failure
packed RBCs
surgical emergency

acute appendicitis

pallor
CBC gram / dl 8


) (
packed RBCs

Hemolytic anemia
written, clinical, X-ray and oral

anemia due to short life span of RBCs

Life span of RBCs
normally 120
life span 119
Hemolytic anemia

diagnostic investigation of hemolytic anemia
subtypes

life span of RBCs

thalassaemia

34


.......................................................................
.............
.N.B
bone marrow
life span RBCs days 60

total count of RBCs
hypoxia
kidney
erythropiotein
erythropiotein stem cell of bone marrow
erythropioesis

RBCs
reticulocytes


Hemolytic anemia

life span of RBCs
days 30
count of RBCs

bone marrow



Life span
RBCs 15
bone marrow
8
bone marrow
life span RBCs
15



manifestations of hemolytic anemia

35


.......................................................................
.............
.N.B

hemolytic anemia
anemia due to short life span of RBCs

hemolytic anemia
clinically presented
Life span of RBCs
15

etiology
hemolytic anemia

RBCs

Intrinsic causes or corpuscualr causes

malaria acquired
RBCs merozyte
merozyte
RBCs
RBC

gene defect hereditary

cell membrane
spherocytosis
hemoglobin
abnormal hemoglobin
hemoglobin
thalassaemia quantity
sickle cell anemia qualitive
RBCs enzymes
G6PD deficiency
pyruvate kinase deficiency

36


.......................................................................
.............
RBCs
extra corpuscular
toxins
plamsa
RBCs toxins
toxins
snake venom
sever infection DIC
toxins
immune causes non immune causes

antibody
transplacental
Iso immune
Intra utrine Rh +ve
Rh negative
placenta
B
A
placenta Anti B

auto immune
isolated RBCs antibody
multi system affection SLE

enumerate 3 different types of anemia caused by 3 different
parasites

iron deficiency anemia
bilharzial and ancylostoma
megaloblastic anemia
diphylopothrium latum

37


.......................................................................
.............
hemolytic anemia
malaria

hemolytic anemia classification

:
acute
chronic
acute hemolytic anemia

Intravascular hemolysis
blood vessel RBCs
toxins
RBCs
intra vascular hemolysis
antibody
intra vascular hemolysis
.N.B
Extra corpuscular causes
acute hemolytic anemia
intra corpuscular
malaria

G6PD deficiency
acute

G6PD deficiecny
Metabolism
free oxygen radicals


H2O2
free oxygen radicals
lipolysis of fat

38


.......................................................................
.............
cell membrane RBCs
lipoprotein
lipoprotein
free radicals
hemolysis of the RBCs
RBCs
RBCs hydrogen

H2O
free oxygen radicals

!!!!

RBCs
glutathione
reduced glutathione
G.SH

reduced glutathione
NADPH
NADPH
RBCs pathway
hexose mono phosphate pathway

RBCs Glucose
glucose kinase

glucose 6 phosphate

glucose 6 phosphate dehydrogenase

phospho gluconate 6

glucose 6 phosphate
phospho gluconate 6

39


.......................................................................
.............
glucose 6 phosphate dehydrognase enzyme
reaction
NADP
NADP glutathione
reduced glutathione
reduced glutathione cell membrane
cell membrane
free oxygen radicals

.N.B


RBCs free oxygen radicals

RBCs
G6PD deficiency
acute hemolysis of RBCs
free oxygen radicals

free oxygen radical

metabolism



free oxygen radical

free oxygen radical

40


.......................................................................
.............








(:
free oxygen radical
drugs


.N.B
G6PD deficiency
antipyretic

sulpha
G6PD deficiency
free oxygen radical

free oxygen radical

free oxygen radical

G6PD deficiency

41


.......................................................................
.............

G6PD deficiency

gene X chromosome
general population typer B+ gene
X chromosome
drak races
type A+ gene
+
G6PD B-ve and A-ve
MCQ
gene of G6PD in dark races
Type A
gene X chromosome
common in males
male XY
female XX

common in males
females

clinical pictures
G6PD deficiency
clinically presented
history of exposure to oxidizing agent
free oxygen radical
RBCs hemolysis
RBCs intracellular content
hemoglobin

free in plasma
intra cellular
extracellular
pyrogenic effect

42


.......................................................................
.............

fever and rigor

RBCs
3

manifestation of anemia
sever pallor
headache, syncopal attack
Manifestation

hemoglobin
free in plasma
hemoglobin
hemoglobin
molecular weight

glomerulus of the kidney
filtration
filtration
acute tubular necrosis & acute renal failure

Liver

high molecular weight
Urine
hemoglobin Hemoglobin
kidney

haptoglobin (a
hemopexin (b
macro protien
Hb Kidney
spleen
spleen

43


.......................................................................
.............
spleen filter
haptoglobin hemopexin
hemoglobin spleen
spleen hemoglobin

hemoglobin extra cellular
very toxic
spleen

hemoglobin

globin and heme
globin
indirect bilirubin
fat soluble
Liver
uptake
liver
direct bilirubin
bile secretion
stool
bilirubin

bilirubin
bilirubin RBCs
hemoglobin haem
RBCs hemolysis of hemoglobin
haem bilirubin bilirubin
haem bilirubin
unconjugated bilirubin ) -1
-2 (
.
44


.......................................................................
.............
kidney liver
kidney
kidney
kidney liver

unconjugated bilirubin liver liver
unconjugated bilirubin
Liver Liver
, Liver Unconjugated bilirubin

receptors Liver Y & Z receptors
unconjugated
bilirubin Liver
Liver uptake
unconjugated bilirubin
conjugation
Liver unconjugated conjugated
conjugated bilirubin ) -1 -2 (
conjugated bilirubin
Urine
secretion
bile bile
intestine intestine

stercobilinogen

intestine
stercobilin
brown stool
stercobilinogen

Liver Liver Liver
Liver
entero-hepatic circulation
45


.......................................................................
.............
kidney Kidney
stercobilinogen urine
urine stercobilinogen
) urobilinogen (
jaundice
urobilinogen Urine

jaundice

Hemolytic jaundice hemolysis RBCs
hemoglobin unconjugated bilirubin
Kidney
Liver uptake unconjugated
bilirubin Uptake conjugated
bilirubin Liver intestine
stercobilinogen stercobilin
urobilinogen
-1 bilirubin
-2 stercobilinogen
-3 stercobilin
-4 urobilinogen
hemolytic jaundice sever
hemolysis bilirubin
hemolytic jaundice

bilirubin hemolysis
Liver bilirubin
urine urobilinogen
stool stercobilinogen
stercobilin stercobilin stool

jaundice Urine
46

stool


.......................................................................
.............

/ Jaundice urine
/ hemolytic jaundice stool
Urine stool
Hepato-cellular jaundice Obstructive jaundice

obstructive jaundice obstruction

Obstruction Liver secretion conjugated
bilirubin bile conjugated
bilirubin
conjugated bilirubin Obstructive
unconjugated normal conjugated bilirubin
urine urine
conjugated bilirubin Urine
obstructive stercobilinogen
Intestine stercobilin
stool urobilinogen stercobilinogen

obstructive jaundice
-1 conjugated bilirubin urine
-2 stercobilinogen urobilinogen
bile bilirubin
bile salts
bile salts
itching bradycardia

itching prostaglandins
Itching unknown
47


.......................................................................
.............
bile intestine
vitamins
cholesterol
jaundice cholesterol
alkaline phosphatase

bile salts Liver biliary cirrhosis

Long standing obstructive jaundice liver cirrhosis

Hepatocellular jaundice
Obstructive jaundice
Hemolytic jaundice
conjugated unconjugated bilirubin
Liver uptake
Unconjugated Unconjugated
bilirubin Uptake Unconjugated
Liver conjugation conjugation liver
excretion
excretion hepatocyte
excretion conjugated bilirubin
conjugated Unconjugated
/ conjugated liver
excretion uptake
conjugated bilirubin bile intestine
stercobilinogen stercobilin
urobilinogen xxxxxxxxxxxx
urobilinogen stercobilinogen

48


.......................................................................
.............
urobilinogen Hepatocellular jaundice
hemolytic obstructive hemolytic
Urobilinogen stercobilinogen
obstructive Hepatocellular
stercobilinogen urobilinogen

stercobilinogen intestine
stool stercobilin
Entero hepatic circulation
kidney urobilinogen
Hepatocellular jaundice stercobilinogen stool
stercobilin Liver Liver

Hepatocellular jaundice excretion

urobilinogen
stercobilin urobilinogen

indirect bilirubin Liver
indirect bilirubin

Indirect bilirubin
Jaundice
free oxygen radical
hemoglobin
hemopexin and haptoglobin

hemoglobin

49


.......................................................................
.............
hemoglobin blood
filtration kidney
chemical tubular necrosis
so early


bilateral
unilateral

hemoglobin urine
hemoglobinuria

Urine
red colored urine
differential diagnosis
oral

Pallor, high grade fever, jaundice, irritable and red colored urine
Irritable Pain


G6PD deficiency history
urinary tract infection
acute pyelonephritis


abdominal examination

examination
Kidney

G6PD deficiecny
UTI
tenderness bilateral
G6PD deficiency

50


.......................................................................
.............
unilateral
acute pyelonephritis G6PD deficiency



urine red in color
hematuria
Hemoglobinuria



Urine
Hemoglobinuria G6PD
) Hematouria ( RBCs opaque
acute pyelonephritis

Investigations
CBC
complete blood count
hemoglobin gram /dl 11
RBCs count
MCV
MCH
normocytic normochromic anemia

blood film
abnormal cells
urine analysis
hemoglobinuria

red color urine

haptoglobin hemopexin

consumed in the spleen
51


.......................................................................
.............

Indirect bilirubin
diagnostic

G6PD assesment
attack
error
blood
MCQ assesment

complications
acute reduction of hemoglobin
anemic heart failure
heart
sever hemolysis
hemoglobin Kidney
massive tubular necrosis
acute renal failure
hemoglobin
gram/dl 5

false screening
HIV hepatitis

treatment G6PD
antibiotic Infection
Packed RBCs
anemic heart failure
acute tubular necrosis
washing kidney

Hyper volemia
congested heart failure

52


.......................................................................
.............
lasix
mannitol diuresis
Urine
Urine output
washing of the kidney

Oral

G6PD deficiency



sever hemolysis
free oxygen radicals

:
destruction of the RBCs

!!!!!!!!!!

G6PD deficiency
resistant

RBCs
Hexose monophosphate pathway
pentose shunt
Pathway glucose 6 phosphate dehydrognase
deficiency

RBCs
hexose monophosphate pathway
text anti malarial drugs
sever hemolysis G6PD
53


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.............

immune modulator
collagen disease
lupus rheumatoid arthritis

G6PD deficiency

Infection


fava beans
metabolism


free oxygen radical
hemolysis


G6PD

free oxygen radical
free oxygen radical
hemolysis

favism G6PD deficiency
G6PD deficiency favism

)

(:

54


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.............
.A smile is a language that even a baby understands
( ):

months 7




clinical manifestation

G6PD deficiency
G6PD deficiency
CBC
diagnostic enzyme assesment

G6PD Normal

enzymatic assay 6
6 G6PD normal


normal
NADP
normal
glutathione
glutathione deficient

chronic hemolytic anemia

life cycle of RBCs

bone marrow
120
cell membrane

trabeculi spleen
55


.......................................................................
.............
spleen channel


phagocytic cells
RBCs
RBCs
hemoglobin

heme globin
heme spleen
Iron
protoprophrin indirect bilirubin
globin

spleen

indirect bilirubin
fat soluble
fat soluble
Liver
Liver Indirect direct bilirubin
Liver
direct bile secretion
GIT
bacterial flora
bacterial flora
stercobilinogen

pathwaies 3
enterohepatic circulation
stool oxidation in GIT
stercobilin
stool
absorped
sysmtemic
56


.......................................................................
.............
stercobilinogen
water soluble
water soluble
Kidney
urobilinogen
urobilinogen
stercobilinogen
kidney

chronic hemolytic anemia

RBCs

15
rate of hemolysis
RBCs count
Manifestation
headache dizziness Loss of concentration
syncopal attack easy fatigability

spleen normal RBCs

splenomegaly
indirect bilirubin
Liver
Liver
indirect bilirubin
Liver
jaundice mild
lower fornices

57


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.............

sclera
liver bilirary system
once jaundice
Liver
Liver hepatomegaly
splenomegaly hepatomegaly

liver spleen
chronic hemolytic anemia
spleen Liver

chronic hemolytic anemia

direct bilirubin
liver 6 direct bilirubin
6
stercobilinogen 6
stool
dark stool

stercobilinogen GIT
oxidation
Stercobilin
stool
dark stool

Urine normal
urobilinogen
urine

bone marrow 8
marrow cavity
58


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.............
skull circumference
Marrow cavity zygoma
Upper jaw
upper jaw


widely seperated teeth
Lower jaw
white marrow
Marrwo
Mongoloid facies of thalassaemic facies
causes of thalassaemic facies

Hyper active bone marrow

chronic hemolytic anemia

gene defect

manifestations of chronic hemolytic anemia
frequent blood transfusion anemia
hepatosplenomegaly splenomegaly
normal urine dark stool
family history mongolid facies
Investigations
CBC
In chronic hemolytic anemia

normocytic normochormic
thalassaemia
microcytic hypochromic anemia

blood film
spherocytosis spherical
thalassaemia anisocytosis target cells
59


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serum iron
Iron binding capacity
Indirect bilirubin 5 /
Liver 6 Normal
stool analysis
stercobilinogen lab investigation
dark stool clinical lab

urine analysis
urobilinogen
X-ray bone
manifestations of hyperactive bone marrow

chronic hemolytic anemia
diagnosis
clinical presentation investigation
specific investigation

general
complications
complication of blood transfusion


hepatitis HIV
spleen
spleen filter
channels
Normal cells
phagocytic abnormal
Normal cells RBCs
phagocytic cells splenomegaly
phagocytic cells
60


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.............
phagocytic cells
splenomegaly
Phagocytic cells
trabeculi spleen
) channels ( tunnel

normal cells
RBCs
phagocytic cells
normal RBCs or abnormal RBCs

frequent blood transfusion


RBCs WBCs
RBCs spleen
trabeculi
cells
WBCs platelets
phagocytic cells
thrombocytopenia Leukopenia

bleeding tendency
frequency of infection
hypersplenism
size phagocytic cells
spleen
trabeculi
normal
frequent blood transfusion
bleeding tendency
repeated infection

61


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.............
Pancytopenia
Liver spleen diaphragm
ribs and throacic cage
normally Liver

spleen

ribs liver spleen
spleen 3 costal margin
chronic hemolytic anemia
spleen
umblicus
abdominal wall
complication
traumatic rupture of the spleen

traumatic rupture of the spleen
rupture spleen
serum iron

transferrin
Iron very toxic

hemosidrosis
hemochromatosis

Hemochromatosis
)(Bronze diabetes
62


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.............

4
hemosidrosis
hemochromatosis
Organs
Organ liver

liver

hemochromatosis
carcinogenic

intestine Mucosal block

63


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.............

deficit mucosal block

6 clinical picture of
hemochromatosis

mental disturbances
hemochromatosis

-1 Liver liver cirrhosis

-2 heart cardio myopathy
-4 -3 hemochromatosis bronzed
diabetes diabetes
diabetes pigmentation


stimulation of the melanocytes
-5 mental disturbances
-6 testicular atrophy

64


.......................................................................
.............
Investigations
investigation for the cause-1

Binding capacity
Investigation for the liver cell failure -2
investigation of portal hypertension -3

Treatment
500 250

250
hemochromatosis
60
56

56 12

repeated venesection iron

chelator desferal

hemochromatosis highly carcinogenic

65


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.............
hepatoma hemochromatosis 30%

Iron

Pituitary gland

pan hypopituitrism
GH TSH FSH
LH
heart
deposit for cardic muscle
cardiomyopathy
.H.F
Iron phagocytic cells
spleen
Liver
6
Pancreas
islets cells

receptor
receptors
glucose metabolism of glucose inside
the cells

hemosidrosis
iron Islets cells

diabetes
normal
diabetes
receptor
gonadal cells

delayed puberty
66


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.............
Infertility
pituitary
skin

Lower limbs
stgnation of the blood
Iron skin
necrosis
ulcers
hemosidrosis
histamine
Itching
ulcers
Liver 6
direct bilirubin
viscosity of bile
biliary stones
gall stone
biliary obstruction
Heart failure

hemosidrosis
anemia anemic heart failure
repeated infection myocarditis heart failure
cirsis

patient with chronic hemolytic anemia

another type of anemia

bone marrow
8
inquirement
iron B12 folic acid
iron
B12
folic acid stores

67


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.............
folic acid
megaloblastic anemia
Crisis
megaloblastic crisis
Over consumption of folic acid
hyepr activity bone marrow
aplastic crisis

thalassaemia
G6PD deficiency
hyper hemolytic crisis
acute hemolytic anemia
on top of chronic hemolytic anemia
Hemolytic crisis

Infection thalassaemia
hyperactive of the spleen
spleen


Phagocytic cells
splenomeglay

complications
repeated infections

WBCs
pancytopenia
heart failure
left sided heart failure
chest infection stgnation of the blood in the lung
hypersplenism
splenoectomy
hemophilus meningeococcal

infection splenoectomy

phagocytosis
68


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.............
chemotaxsis organism
immune system

immune system Phagocyte Organism
cell migration

phagocyte organism

cell adhesion
phagocytosis
enzyme
intra cellular killing of organsims

capsulated organism
phagocyte
capsulated organsim

chemotaxsis
phagocyte Organism
capsulated oragnism

capsule
very smooth
surface

Phagocyte
phagocyte can't adhere to capsulated organism
spleen opsinisation
glu like material
specific for capsulated organsim
complications
Pathological fracture
pathological fracture
trauma
fracture

69


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.............
cortex
medulla
trauma fracture

complications
stunted growth
short stature why
hormones
.G.H
hypopituitrism
hemosidrosis
somatomedin
TSH
T3 and T4
Insulin
One of the main growth factor

repeated infection

growth hormone
receptor
receptor
Somatomedin
receptor .G.H Somatomedins

chronic hemolytic anemia

treatment of anemia
bone marrow abnormal cells
spleen
bone marrow transplantation

packed RBCs

70


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.............

ordinary transfusion


gram /dl 6
hemosidorosis

hemoglobin gram /dl 8


dysmorphic features
hyper active bone marrow

hypertransfusion

hemoglobin gram / dl 10

hemoglobin 3 4

hemosidrosis


dysmorphic features

supertransfusion

hemoglobin gram /dl 12

oxygen supply

folic acids

Hyperactive bone marrow

71


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.............
treatment of the complications
hemosidrosis
iron chelating agent

how to diagnose hypersplenism clinically

history of frequent blood transfusion
bleeding tendency
repeated infection
CBC
pancytopenai
to be sure of hypersplenism
250
hypersplenism
250


9/1/2012 (:

9/1/2011


say 12
12000

40
12000/40
ml /kg per year 300

sever hypersplenism
hyper splenism
spleen
spleen capsulated organsim

72


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.............
meningeococcal pneumococcal vaccines
salmonella and hemophilus infeluenza vaccine

booster dose


splenoectomy

spleen

vaccines of capsulated organsims

splenoectomy spleen
spleen
antigen processing centres

traumatic rupture spleen
vaccines

long acting penicillin

spherocytosis

RBCs
biconcave in shape
small capillary
small trabeculi of the spleen

73


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.............
RBCs Life span
120
spleen

spleen RBCs 120

RBCs ATP
Pump 120
120 ATP
Na pump


RBCs spherical in shape
RBCs trabeculi spleen
trabeculi
phagoctytic cells

RBCs biconcave
) (
RBCs
Na mainly
extracellular




!!!!
main gates



spectrin
neutral protien
positive charges
protien

74


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.............
RBCs

small gates amino acids
glucose

Main gates

RBCs

Na pump
active pump
ATP
Na pump ATP RBCs
RBCs

spherocytosis
spherocytosis autosomal dominant gene
gene defect
Positive family history
autosomal

male female
no sex difference
dominant

spectirn

main gates
RBCs
Main gates
side gates
Na pump
ATP
75


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.............
Pump


spherical in shape RBCs
spleen
phagocytic
hemolysis so early

channel
main gates pump

clinical picutres of spherocytosis

positive family hisotry
no sex difference ( autosomal dominant)
hemolysis age of onset
hemolysis
Liver
still immature
bilirubin
bilirubin

neonatal jaundice

Indirect bilirubin

fat soluble
blood brain barrier
kerniectrus bilirubin encephalopathy
kernicterus
cerebral palsy

spherocytosis clinical picutures

general clinical pictures of chronic hemolytic anemia
anemia not responding to treatment
frequent blood transfusion
76


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.............
splenomegaly then hepatomegaly
jaundice
dark colored stool , normal urine
8-7

spherocytosis neonatal jaundice
physiological jaundice


physiological jaundice

physiological Pallor Pathological Pallor
Physiological organomegally Pathological
physiological general condition
Pathological

complication of spherocytosis
complications of chronic hemolytic anemia
gall stones

chronic hemolytic anemia

Investigations
general invetigations
CBC
Anemia
normocytic normochromic anemia
Normal MCH MCV

spherical in shape
poly chromisia

RBCs
RBCs
blood film
77


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.............

RBCs
spherical

RBCs

RBCs
serum iron
iron binding capacity
indirect bilirubin

liver consumption
Neonatal period
stool analysis
stercobilinogen
urine analysis
urobilinogen
X-ray bone
chronic hemolytic anemia
diagnostic investigations

Osmotic fragility test


test tubes
variable concentration of Na

Normal saline 0.9
0.9
RBCs
RBCs saline
spherocyte
0.2-0.3-0.4-0.5-0.6-0.7-0.8

78


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.............
RBCs
RBCs
RBCs

Biconcave RBCs Na 0.9


0.8
RBCs 0.9
) 0.8 ( saline
RBCs



0.6
normal 0.5

hemolysis
0.3
No cells
deposit
hemolysis complete

0.5 complete hemolysis 0.3

spherocytosis
RBCs
0.9 RBCs
0.8 RBCs
0.7 deposit start hemolysis

0.5 No cell

hemolysis
79


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.............
increase autohemolysis of RBCs
test tube
normal saline 0.9
deposit RBCs
normal saline
24


deposit
RBCs hemolysis
RBCs hemolysis normal saline
24
RBCs
RBCs Biconcave shape
120

autohemolysis spherocytosis

spherocytosis

glucose 24

RBCs
RBCs
diabetes spherocytosis

packed RBCs
folic acid 5 mg/day
iron chelating agent therapy

cholecystomy
gall stones

80


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.............

spleen
spherocytosis
spleen spherocytosis
hemolysis
RBCs 120
other reticuloendothelial system

trabeculi
spleen hemolysis
CBC
blood film
spherocytosis
clinical
laboratory

splenoectomy !!!!
spleen
is the main mature part of the reticuloendothelial system in the first
5 years of age
lymph nodes immature
Maturation
Lymph node Mature
spleen
booster dose
spleen
spleen


frequent blood transfusion
folic acid
complications
spherocytosis
RBCs
ATP
81


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.............
spectrin
spectrin

direct indirect bilirubin

direct
blood
biliary system

blood
Liver
direct bilirubin sclera
water soluble Urine
lemon jaundice
urine
direct bilirubin blood

Indirect bilirubin
fat soluble


golden yellow
Liver
Urine
Urine

Urine
indirect hyperbilirubinemia

urine
direct hyperbilirubinemia

RBCs spherical

spleen trabeculi lymph nodes
82


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.............
phagocytic cells

, RBCs
spherocytosis
spleen
thalassaemia bone marrow transplantation
sickle cell

glucose Normal person

glucose

thalassaemia

Normal hemoglobin abnormal hemoglobin

normal hemoglobin
soluble in cytoplasm
soluble in cytoplasm

hemoglobin chains 2
alpha chains
protien part hemoglobin
chains 4
alpha chain hemoglobin
Alpha chain 16
alpha chain

thalassaemia
quantitative defect
83


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.............
In chain synthesis
in protien part of hemoglobin
alpha chain thalassaemia
alpha thalassaemia
alpha thalassaemia clinical
.i

.ii

.iii
.iv

deletion 3
alpha chain silent carrier

gene maping

2gene defect



alpha chain
defect
alpha chain
hemoglobin

sever heart failure

2chains
Hemoglobin
chain 2
gama chains
alpha and 2 gama 2
) fetal hemoglobin ( Hb F
fetus
chain chromosomes
11
Genes 2
Gama synthesis

Beta chains

84


.......................................................................
.............
Hb A adult hemoglobin
Beta chain gene 2 11
dominant
thalassaemia
Beta thalassaemia

Beta thalassaemia minor
Beta thalassaemia major
delta chain
Alpha chain
adult hemoglobin
Hb A2
11

blood
PNH
PNH blood element blood
elements RBCs platelets WBCs
cell
membrane RBCs platelets WBCs
in activation active
complement complement activation blood
activated complement blood RBCs
WBCs platelets
cells
complement
In activation
RBCs active complement
complement activation complement
activation Hypoxia
hypoxia ... partial hypoxia
Hypo ventilation
... respiratory rate shallow
breathing
respiratory rate adult 18 10
...... Hypoxia hypoxia PH
85


.......................................................................
.............
7.35
PH activation for the complement
activation for the
complement
blood elements

hypoxia
PH 7.4 7.35
PH ... complement activation
activation
fever Loin
pain Intravascular hemolysis
hemoglobinuria
paroxysmal attack
nocturnal Hemoglobinuria
paroxysmal nocturnal hemoglobinuria

CBC pan cytopenia
acidosis acidosis
metabolic or respiratory acidosis
acidosis complement activated
pancytopenia
PNH
PNH Ham test
Ham test acidic media
acidic media complement activation
blood elements .... Ham test
acidosis acidic media
diagnostic for PNH

86


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.............

**** !!!!!
"..... ,
" *****

87