Professional Documents
Culture Documents
With
Prof. Dr Mohammed Abo El-Asrar
Edited By
El-Azhar Medical students 2012
Hematology
MCQ X-Ray hematology
RBCs Anemia
Platelets bleeding disorders
WBCs
Anemia
-1
-2
-4 Investigations
Definition
Pallor ) Pallor Pallor (
Hb < 11 CBC
18
4 1- Neonatal period
2- Infant
12 10 3- Childhood period
Adolescent 4- 18
: Fetus
1-Fetus in Intrauterine life
2 fetus Hb is Hb M which has No good O2 dissociation
fetus Partial hypoxia
O2 sensors PO2 hypoxia
fetus ) Erythropoietin (
RBCs synthesis liver &spleen bone marrow more RBCs
normal Hb 18-22 gm.%
lung hypoxia Erythropoietin Hb 9 15 14 12
10 6 ) ( gm.%9
physiological anemia of the newborn
hypoxia BM RBCs
! Iron !!
MCQ: - cause of anemia in newborn
1-haemolisis of RBCs 2-nutritional cause 3-bleeding tendency 4-decreas Erythropoietin level
(Causes Aetiology)
: Decrease RBCs Count
1- Decrease Synthesis as in:
1-BM problems BM failure (hypoplastic Anemia)
RBCs Erythropoietin H
4-Infeltration of BM
stem cells
5-may be Liver & kidney diseases
decrease Erythropoietin H or other hormones.
6-Irradiation.
Investigations
CBC .......
: CBC
-1
11gm % ......... HB
-2
CBC
:
1- Mean corpuscular volume (MCV) RBCs :
80 +/- 20 femto liter = RBCs
Macrocytic anemia
Definition :
- defective synthesis due to iron
iron metabolism
-Daily requirement 2-3mg/kg/day
-animal sources
simple form so complex IDA
HCL FERROUS STOMACH FERRIC IRON . IRON ABSORPTION ACIDITY
- ARTIFICIAL MILK
- But BREAST MILK NUTRIANT in reaction so, NO CHANGE IN PH so , ALL IRON of it is absorbed
& ARTIFICIAL MILKALKALINE IN REACTION DECREASE ACIDITY so, ABSORPTION OF its IRON
NSAID may ulcer ANTACID IDA
ALKALINE may IDA
-
- ABSORPTION occur IN THE UPPER PART OF THE DUDENUM <ACIDIC part> DEPENDANT ON a
CARRIER PROT. <APOFERRITIN>then CONVERTED TO FERRITIN TO CARRIIER PROT IN BLOOD
NB. apoferritin + iron = Ferritin
Transferritin ( ferittin in blood) to stores in liver
.NB ) (
HEMOSEDIROSIS DEPOSITION in tissues FREE
-
.. common
6 breast iron stores
breast stores 6.
6 iron
. 9 called DELAYed WEANING
CAUSES OF IDA
either
1.INTAKE:
Signs:
1-general signs. As before
2-specific signs.
1-red glaze tongue
2-spooning of the nails atrophy
3-10-15% spleenomegaly
Investigations
1-CBC
1-anemia or not Hb < 11 gm %
2-type microcytic ( MCV < 60) hypochromic ( MCH < 26) anemia.
even reched zero.
retigulocytic count
3-Cause:
synthesis
Fully saturated 3 transferrin iron binding capacity ( transferrine ) serum iron (. transferrine ... )
iron binding capacity 3 iron saturated
heam
Or discuss D.D ??
1- iron deficiency anemia *
2- thalassemias *
3- lead poisoning
4- sidroplastic anemia
D.D
...
1- I.D.A
- retics
- blood film no abnormal cells
- serum iron & iron binding capacity
2- thalassemia:
- retics loss
- blood film
-Hb.electrophoresis Hb.F
3- lead poisoning:
TTT of IDA
1- prevention:
1- antenatal iron therapy
iron therapy stores2- proper breast feeding:
severe gastritis oral partenteral gastritis
oral iron
2-oral:
iron elemental
....
4 3 !!!
inbetween meals
72 reticulocytic count
0.1 3 0.5
Hb .
9.5 9 11 3 13 12 14 4 6 stores
- common side effect
stool dark stool iron iron
? when
3- packed RBCs
:
6mg% or less
1-Hb
Haemolytic anemia
Def :
anemia due to short of life span of RBCs normaly 120 days
119
????????? diagnostic investigation of haemolytic anemia
.. life span
reticulocytes radio isotopes .. 15
haemolytic anemia
- 60 ) life span ( hypoxia
erythpiotin
8 normal
BM 8 ... 15 So, clinical present only if life span decrease blow 15 dayes
)Causes (etiology
1- intrinsic cause = Corpuscular cause
- Problem in RBCs
1- Acquired :.
- As malaria merosoite enter RBCs RBCS 8
2- Hereditary :.
- Cell membrane Spherocytosis
- Hb abnormal :. Either Quantity Thalassemias or Qualitaty Sickle cell anemia
- Enzymes G6PD deficiency or pyruvatite kinase deficiency.
2- Exra-corpuscular = extrinsic cause
1- Toxins = non - immune cause
- Snak poisons
- Endogenous toxine ( sever infection or DIC )
2 - Antibodies immune cause
- Transplacental
Rh incompatibility or ABO incompatibility
- By blood transfusion B O in which serum contain Anti-B
O ....
- Autoimmune Antibodies against RBCs
either Isolated ( aginst RBCs only ) Or aginst all systems as :. SLE
1- Ancylstoma
3-
4- megaloblastic anemia
Classification of H. anemia
1- Acute:
- Intravascular haemolysis blood vs. as toxins & antibodies ( G6PD intravascular)
- Intacorpuscular malaria
2- Chronic : ( inside the spleen Extravascular )
thalassemia, sickle cell anemia & spherocytosis
G6PD
:
. glucose 6 phosphate dehydrogenase
Free O2 radicles ( H2O2 or O3) lipoprotein RBCs cell membrane lipolysis of fat (O3) O2 H2O O H2 RBCs RBCs
reduced glutathion H2 -
Causes of G6PDD :.
)(X-linked receissive gene defect
chromosom X receissive gen - general of population Called type B+ Enzyme synthesis
black races . type A+ genetics A delated gene B- MCQ x common male Xy male Xx female
X male X X
X ) X (
female .
clincal picture
1-history of exposure to oxidizing agent that relase free radicles.
...... infection free o2 radicles
RBCs Hb free intra-cellular extacelluar pyrogenic effect
2- so, fever & rigors:
fever & rigors 3- manifestations of anemia :
RBCs manifistation of anemia sever pallor acute
4- jaundice
Hb ) molecular weghite (M.W free gloumeruli acute renal failure plasma proteins high M.W liver Hb
kidney ) acute tubular necrosis plasma protein urine High
(M.W
plasma protein:1- hapatoglobine
2- hemopectin
both macroprotein take Hb - kedney
- spleen Hb ) (2&1
Hb is very
toxic
Hb globine & haeme indirect bilirubine fat soluble liver direct bile
indirect liver )( indirect5- lion pain:
free O2 Hb ) (2&1 free kidney chemical tubular necrosis
so early loin pain bilateral unilateral .. 6- red color urine :
Hb Hburia urine Hb .7- More in male than female :
:
high grade fever ,pallor ,jaundice , ) irritable (pain
activity
:
1- G6PD
2- urinary tract infection pyelonephritis hematuria
.
Abdominal Examination
NB: pain
tender kidney G6PDD bilateral
-
Hb uria ) (
opaque
Investigation :
1- CBC
- Hb < 11 gm%
- MCB ,MCV = normal So, normocytic normochromic as other RBCs are normal.
- RBCs more erytropoitine reticulocyte So, retics ++
- Blood film
2- Urine analysis: Hb uria
3- Haptoglopine & Hemopectine
4- indirect bilirubine
intra vascular Hemolysis diagnostic Enzyme
)6- G6PD Enz. Level : (DIAGNOSTIC
attack 6 ) (
Complications :
Hb14 gm % Hb 7 gm % acute
1- anemic HF
2- acute renal failure
3- complications of Bl. Transfusion :
-
HB 5gm% infection
Hepatits & HIV
ttt :
: + ttt of infection 1- Avoid precipitating factors
2- Packed RBCs
anaemic HF
acute tubular necrosis 3- Washing of the kidney
hypervolemia
, lasix washing of the kidney uine output
:
/ .. merzoite RBCs
pentose pathway
RBCs 120 .
severe jaundice
biliary system liver 5- hepatomegaly normal 6 liver
spleen liver spleen
6- also, dark stool due to more sterchobillin ( mother complaint )
7- but urine is normal
8- mongoloid features or thalassemic features:
: marrow cavity normal 8 BM - In skull , prominent upper jaw ( so, widely separated teeth ) but lower jaw contain white marrow (
)
Also , prominent zygoma
mongoloid features
thalassemia tahalssemic features
. 9- family history:
genetic -
Investigations
1-CBC :
normocytic normochromic Except Thalassemeia mictocytic hypochromic + Reticeulocytes
2- Bl. Film
spherical shaped RBCs spherocytosis
- or sickle shaped RBCs. sickle cell anemeia
- or anisocytosis & target cells. thalassemia
3- serum iron + IBG
4- indirect billrubin
not > 5 mg/dl ( due to liver
liver compensation )
5- stool analysis sterchobillin.
6- urine analysis urobillinogen.
7- X Rays
6 5
1-
2-
with infection
capsulated organisms
pneumococci, H.influnza, meningococci, salmonella, etc
incidence with capsulated organisms
cell migration chemotaxis phagocytosis
digestive enzymes cause intracellular killing
resist phagocytosis capsulated organisms
phagocytic cells capsulated organisms
capsule is very smooth
capsulated organisms glue like materials -opsonins spleen
( ) phagocytes < --organism
. spleen
9- Pathological fracture.
expected truma < ---physiological fracture non expected trauma pathological fracture ( )
. truma medulla cortex 10- stunted growth. (
)
)
1- Endocrinal:
1- G.H.
2- somatomedins due to G.H.
as G.H. somatomedins.
3- (T3 & T4) hypothyroidism
4- also insulin D.M.
NB. G.H. need somatomedins receptors
2- Anemia No good oxygenation.
3- Chronic toxaemia .
4- Pathological fracture.
) (
2- Hypertransfusion.
3- Supertransfusion.
12mg ) (
2- Folic acid :
B.M. 8 5mg/day folic acid
3- Treatment of complications:
1- Hemosidrosis.
)(iron chelating therapy
Parenteral
) (75
)
) (growth 5.
)Oral (under trials
vaccination ..
spleen antigen presenting cells markers
spleen
emergency traumatic rupture of spleen long acting .penicillin 6 7
Spherocytosis
introduction
) (
:
biconcave RBCs
small capillaries and small trabiculae
1
* 120 spleen 2
: 1 )(biconcave
cytoplasm .
mainly extracellular
Etiology
autosomal X +ve family history autosomal dominant gene defect
1:1 male or female
main gates Na ( abnormal)
Na ATP Na-pump
spleen spherical shape
(...... 10 5 )
( ) main gate :
Clinical picture:
1- +ve family history
2- No sex difference
3- Age of onset : since birth
RBCs
hemolysis increase of bilirubin & the liver still immature leading to neonatal jaundice with indirect bilirubin
that may cross BBB leading to kernictrus= bilirubin encephalopathy
4- General c\p of chronic hemolytic anemia
- not responding to hematinics ttt , Increase frequency of blood transfusion & hepatosplenomegaly & dark
stool & normal urine , Dysmorphic features ( thalasemic features)
spherocytosis general c\p
Complications :
As all hemolytic anemia + complications of neonatal jaundice ( kernictrus)
+gall stones
.. hemolytic
investigations :
1- General investigations:
1- CBC :
normocytic normochromic anemia & retics increased & blood film is sphericalRBCs
+ polychromesia
2- Serum iorn increase + decrease TIBC
3- Indirect bilirubin
4- stool analysis
RBCS
RBCS
0.9 0.8 Na .. RBCs 0.6
0.5
so start hemolysis normally at 0.5
.. 0.3
So complete hemolysis normally at 0.3
spherocytosis 0.7 so start hemolysis at 0.7
0.5 cells
So complete hemolysis at 0.5
2- Autohemolysis:
) ( ) (
0.9 RBCs 24
RBCS
RBCS
spherocytosis more dark
-
RBCS biconcave
DM DM
TTT
1- packed RBCs
3- iron chelating therapy 4- ttt of gall stones
2- folic acid
5- spleenectomy
Spleen complete clinical cure
laboratory RBCS RES trabeculae
spleen 5
infections LN
consent .
:
spherocytosis hemolysis
.. ATP
.. 10 15
spleen RBCS spherical ..
THALASSEMIA
Introduction
normal Hb abnormal Hb normal Hb soluble in cytoplasm
part Hb Hb alpha chains 2 16
-thalassemia
-thalassemia means quantitative
quantitative defect in chain synthesis of protein part of Hb
Hb
alpha alpha thalassemia 1- deletion of one gene :
deletion of one gene 3 silent carrier ) ( gene study2- deletion of 2 genes:
2 3- deletion of 3 genes
3 .4- deletion of 4 genes :
4 Hb abnormal RBCs anaemic HFs ) (hydrops fetalis
chains Hb chains Fetal Hb = Hbf
1- if chains 2 + 2
so, no thalassemia
) HbA (adult
: 1- B thallasemia minor
2- B thalassemia major
3-delta chain with make Hb A2
- gene on 11 chromosome
8 7
normal : 1- intrauterine:
itrauterine 6 cord blood - Hb F 70 % of total Hb & 30% of RBCs Hb A
RBCs spleen and liver
2- after delivery
no changes
3- after 6 months
- Decrease in Hb F + increase in Hb A
- To 1 year Hb F reach
1% , HBA
: curve
- chain (gene) constant work since intrauterine life until death
- chain (gene) intrauterine show maximaum activity till 6 months then decrease till
one year
- B chain gene
B thalassemia
B chains genes 2 11 B gene B thalassemia gene minor one gene of 2 genes pathological gene single gene defect
- Pathological gene is a ressive gene & the other normal Gene is a dominant gene
- so, geno type of thalassemia minor is Rr {heterozygos}
(R normal dominant gene
2- > 6 months:
% ................ % - % -% - % activity gene 6
. HB - So the onset of B thalassemia major 6months
But spherocytosis since birth & G6pD .D any time exposed
B thalassemia
Due to ineffective erythropoiesis Not hemolysis
Hb RBCs normal Hb
BM
hemolysis
maximal activity RBCs 2chain B 10 RBCs 20chain defective erythropiosis only 1 RBCs
hemolysis
pure Hb 18 - Which is insoluble Hb that deposit on cell membrane of RBCs cause intramedullary hemolysis
- Some of them get out from BM bizar shape RBCs (abnormal shape)
: ................. 3 4
target cells & anisocytosis (( ) diagnostic Bl.film) spleen hemolysis
only target cells ( which is non functioning cells) extramedullary hemolysis
pathophysiology 3 .. 1- onset > 6 months gamma activity
2- ineffective erythropiosis
- Also , microcytic hypochromic anemia
3-hemolysis:
Hemolytic 1- intramedullary hemolysis in BM 2- extramedullary hemolysis in spleen
Clinical manifestations
1- age of onset > 6 moths
2- female = male as its gene is autosomal
3- anemia not responding to hematinics 6
4- history of frequent Bl. Transfusion , jaundice , stool (darker) , no urine change , thalassemic features
due to hyperactive BM Or presented with complications etc
general -
Investigations:
- CBC microcytic hypochromic Anemia + retics , Bl film target cells + anisocytosis
- serum iron + IBG .etc as before.
+ X-ray on bone hyperactive bone marrow
+ Diagnostic Hb electropheresis
HbA 96%
- HbA2 3.2%
- Here :
RBCs %100 Hb A 0% - only Hb A2 & Hb F ( mainly Hb F
) electropheresis test ( alkaline denaturation test o2 dissociation HB F test
+ Antenatal diagnosis
Treatment
1- packed RBCs
2- iron chelation not before 5 years .etc as before
B THALSSEMIA MINOR
SINGLE GENE defect (Rr) heterozygous
:
.. :
10 RBCs RBCs
5 RBCs HbA 60% Hb = 9-9.5
so, no severe anemia as it is compensated
So , No hepatosplenomegaly only pallor called carrier
carrier
iron deficiency anemia :
1- CBC
Hb = 9-9.5 , microcytic hypochromic + reticulocytes
2- Iron level
) IDA ( iron + iron binding capacity
3- Hb Electrophoresis :
- in normal HbA:HbA2 = 30:1
A A2
- Here 20:1
Treatment
HEMISIDROSIS
.. MAJOR
:
1-follow up of iron level in blood.
tannic acid 2-After meal
Alpha thalassemia
4 alpha 16
alpha thalassemia
1-single gene defect
silent carrier
single gene defect carrier
. silent
Hb electrophoreses
target cells 4 chain Hb which is insoluble called Hb- Barts (4 gamma chain) 6
6
Hb H ( 4B chain)
B-Major 4- 4 genes defect hydrobs fetalis
. 6 Hb-H Hb-Barts 9 8
or
Insoluble hypoxia
B chain
sickle anemia
- if artery:
either infarction or ischemia pain
So, this attacks called painfull crises or vasovaso-occlusive crises
- if vein :
spleenic vein .. tributaries
filter spleen %20
Marked congestion in spleen its capsule has sensory fibers if stretched severe pain
then syncopal attack..
WHY SYNCOPAL ATTACK ???
Due to :
1-vaso-vagal attack due to severe pain.
2- COP as 20% of blood in spleen severe hypotension hypovolemic shock.
: 1-sever pallor
+ blood film
O2 sickle cells
Diagnostic :
1-sickling test
na-metabisulphide O2 RBCs
Treatment
attack
1-vaso occlusive crisis :
)- stop ppts factor (stop sickling
- -
- analgesic
- exchange transfusion canula canula
transfusion
Bleeding Tendency
. 3 :
: Bleeding tendenacy
:
:
Q1:
1- massive uncontrolled bleeding
bl. Tendency
2-bleeding from one orifice
systemic cause local cause3-from two non-repeated orifices
epistaxis ) Called 2 repeated orifices (false hematemesis
bleeding gums hematuria 2 non repeatant orifices4-uncotrolled bleeding after minor trauma
.. hemtoma bleeding tendency
5-or after minor surgery
circumcision 40 liver coag. factors
.. 40 ) (hematology
- menstruation
Q2 cause :
bl v trauma ... bleeding :
bleeding
blood flow
1-local v.c
2-platlets:
purpura ) ( -
insect bite -
3- ecchymosis:
ecchymosis echymosis multiple ecchymosis hematuria epistaxis
platlet & VC minor injury
co-agulation -
2-autoimmune SLE
3-vit c difciency
4-sreroid
vessels support collagen 5- meningococcal septicemia
Platelet causes:
causes:
1- platelet count (thrombocytopenia): normal count: 150.000-400.000/mm2
or
2- defect in platelet function
Causes of thrombocytopenia:
production
1.
2.
3.
Toxins Drugs Irradiation - Viral infection ( HPV, HBV, EBV) - Abnormal metabolites - Infiltration
Excessive destruction:
as in:
2- in DIC
defect in coagulationintravascular thrombuswhich is destroyed by fibirinolytic systemformed againdestroyed
consumption platelet< --
4-Kaselbach-merritt syndromehemangioma
platelets
10 9
which is rapidly absorped reach bloodcause activation of coagulation cascade form thrombus
consumption of platlets fibrinolytic system
Thrombothenia:
platelet
platelet adhesion
receptors platelets injured BVs wall Von willibrand factor (type of plasma protein) *
glycoprotein Ib receptors ) ( glycoprotein ... VWf
cell membrane
1-VWF
2-receptors
adhesion *
: thrombothenia
1.
Acquired: cyclooxygenase
2.
Hereditary:
Coagulation disorders:
purpera or rash .. circumcision < --
Only echymotic so, defect in coagulation factors.
extrinsic pathway , intrinsic pathway & common pathway
1-Extrinsic pathwayonly factor vii then activation of common pathway
2-intrinsic pathwayfactor xiixiixviiicthen activation of common pathway
3-common pathwayxiii(fibrinogen to fibrin)
defects
1-hereditary defects:
- no factor vii
- intrinsic pathwayviii, ix or xi hemophilia which has 3 types:
b. ix
a. viiic
c. xi
- Consumption of fibrin:
Investigations:
bleeding tendency
hematuria with no urinary affection
:
Vascular- thrombocytopenia-thrombothenia-coagulation system defect
minor injury
bleeding time ... bleeding
prolonged MR bleeding time purpura
* Normally bleeding time 60 sec.- 5 min. (range )
prolonged > 5 min.
15 CBC ..vascular or platelet minor injury
thrombocytopenia 100.000 platelets counts **
400-200 BM aspirate
- if impairedthrombothenia
- If normalsure vascular
(prolonged bleeding time, ITP :
platelet, BM: megakaryocyte)
N.B: vascular causes known by exclusion
:If bleeding time is normal coagulation system defect
Vii, xii, xi, viiicfactors
or
2cm
partial Reagent (activate factor Xii) + stop watch till formation of thrombus
thromboplastin time PTT, normally: 25-40 sec.
Reagent(activate vii) and calculate time till thrombus formation prothrombin time PT, normally: 12-14
sec.
(so,normal intrinsic) normal
30 PTT -
hemophilia a or b or c c. xi
b. ix
a. viiic
prolonged 30 PT
Common pathway or vit. K (not DIC prolonged bleeding time)& not liver cirrhosis
Henoch-schonleinpurpura
drug viral allergic vasculitis 2:1 - At any age but more common 2-8 years
2 2 :
1- Extremities purpura
extensor surface of the forearm buttocks L.L special distribution.
palpable odema allergic ) ( purpura :
( L.L ) -
+ itching
2- Joint affection : Arthritis and arthralgia
Red,
Red, hot,
hot, swollen,
swollen, not
: non essential
1- Acute glomerulonephritis or any for
form of renal affection (nephritis
nephritis)
ritis)
2- GIT vascularitis Abdominal colic and diarrhea
intusussciption loops -
: investigations
- normal CBC
- platelets : normal
: Complications
1- intusussciption 2- Renal failure
TTT:
- As any allergic : self limted
- may give steroids ( low dose ) with or without anti histaminic
if Joint affection Never give Aspirin
Give another analgesic Bleeding
11 10
Investigations:
prolonged bleeding time purpura -1
100,000 platelet CBC -2
. mega karyocytes B.M -3
*B.M aspiration is mandatory to exclude serious conditions & malignancy
TTT:
self limited platelets immune CBC
- if no clinical ( no active bleeding ) & if Platelet count > 40.000 just follow up
- CBCevery week or 2 weeks
(IC hge ( ) serious hge) 40.000 1- So, give immune suppressive as predinsolone .
2mg/kg/day_ max: 6mg/kg/day : ( )
chronic ITA :
6 Female SLE EVAN syndrome HIV
platelet sever bleeding
Hemophilia
3 types :
- Hemophilia A: deficient factor VIII - Hemophilia B: deficient IX - Hemophilia B: deficient factor XI
- Inheritance:
- A&BX-linked
resessive. - C Autosomal resessive. - so A&B more in males and C : Both.
A&B
after delivery:
bleeding from the umbilical cord , after circumcisin or after IM injection of vit.k ( which is a routein)
then: Bleeding
- multiple echymosis without petiche or purpra
-minor trauma : hematoma
-severe bleeding on minor injury
sever bleeding - hemoarthrosis
( ) hemoarthrosis stiffness of joint . fibrosis ( bleeding inside joint)
- sub periosteal He:
healing by fibrosis the sub periosteal Hge trauma muscles Bone calcification
Hemophilic pseudo tumor tumor long life diseases -
complications :
1- ICH (serious Hge )
2- complication of blood transfusion
3-hemoarthrosis lead to stiffness of joints .
4-hemophilic pseudo tumor.
5-factor replacement for life:
( ) factors ABS (inhibitors )
investigations :
1- bleeding time:
normal Bleeding time petichea or purpura 2- PT for extrinsic factors here, normal
3- PTT for intrinsic factors intrinsic
4- then Factor assay
:severity Factor - mild if 6-30% of normal
- moderate 1-5%
- severe < 1%
TTT
1- avoid precipitating factors:
trauma .. bleeding 2- factor replacement replacement therapy
Thrombathenia
. 3
:
platelet functions : ADP reagent : 2 - .restocetin reagent : 1 glycoprotein 1B receptors VW F wall of blood vessel Restocetin .platelet
. platelet IIb + IIIa ADP :
. VWF 1b restocetin : Glanzmann`s
But not around restocetin No VWF or Ib add VWF if aggregate is VWF disease.
Bernerd soulier syndrome
1- VW Disease
b b kidney b b b b urine b LMW protein in intrinsic pathway Viiic liver
b Viiic VWF kidney carrier macroprotein VWF
hemophilia A
PT normal PTT - Viii lost
prolonged Bl. Time function platlet adhesion VWF
+ petichea & purpura + normal ADP
+ with restocetin No
VWF
- ttt : as hemophilia A
2- Bernerd soulier sundrome
sundrome
- No glycoprotein ib receptors no platlet adhesion .
- bleeding time increased , normal with ADP and impaired with restocetin (not corrected)
- CBC thrombothenia .also , giant platlet ( platlet
glycoprotein 1b contraction in wall of platlet decrease its size
spleen thrombocytopenia
3- Glanzmann`s disease
deficiency of vit. K
- causes:
1- decrease intake .
2-vit. K is fat soluble need bile
3-decrease in bact. Floora. due to prolonged use of Abs for more than 1.5 months
- bleeding time normal , increased PT and PTT
NB. if active bleeding vit.K 72 give FFP ()
liver Dis. ( )
Multi System disorders Bl. Transfusion leckocytosis pancytopenia DIC NB. VIII inhibitors plasmapheresis .
. 11 1:27