Bleeding Disorders of

Primary Hemostasis
Introduction
 The abnormalities in hemostasis resulting in bleeding
arise from defects in one of four processes:
1. A deficiency of platelets ( The most common causes).
2. An abnormality in the coagulation proteins (the 2nd
commonest cause).
3. Abnormal platelets function.
4. Vascular abnormalities (rare).
Disorders Of Platelets
Classification
 Quantitative disorders
Thrombocytosis vs. Thrmobocytopenia
 Qualitative disorders
Abnormal platelets function:
1. Congenital
2. Acquired .
Thrombocytopenia
 Decreased in platelets count in PB to a level below the
expected lower range( < 150 X 10³/ul ).
 Clinical presentation:
1. Spontaneous skin purpura.
2. Mucosal hemorrhage (as epistaxis & gingival
bleeding).
3. Prolonged bleeding after trauma.
Causes of thrombocytopenia
1. Increased platelet destruction
 Immune mediated
• Autoimmune
- Primary: Immune thrombocytopenic purpura
- Secondary: SLA,B-cell lymphoid neoplasms
• Alloimmune: Post transfusion purpura
• Drug-induced: Heparin, Quinidine and sulfa compound
 Non immune mediated
• DIC
• Thrombotic thrombocytopenic purpura, Hemolytic uremic syndrome
• Mechanical destruction: Heart valves
• Microangiopathic hemolytic anemia.
2. Decreased production of platelets
• Generalized primary BM diseases: Aplastic anemia
• BM invasion/infiltration : Acute Leukemia
• Selective impairment of platelet production:
- Drug-induced: Alcohol, thiazides, Cytotoxic drugs
- infections: Measles,HIV
- Ineffective megakaryopoiesis: Megaloblastic anemia,MDS
3. Increased sequestration:
• Hypersplenism
4. Dilutional
• Massive stored blood transfusion
 Pseudothrombocytopenia:
o Artifactual low platelet count due to in vitro clumping
or satellitism should be excluded!!
o Platelet clumping usually caused by antibodies that
bind platelets only in presence of chelating agent
(EDTA).
o "Platelet satellitism" - platelets stuck to WBC ( Most
common to neutrophils).
Platelet clumping in EDTA No clumping in heparin
Platelet “satellitism”
Thrombocytosis
 Increased platelets count in peripheral blood to a
level greater than expected upper normal range
( > 450 X 10³/ul)
 Mostly occurs due to increase platelets production in
bone marrow.
 In splenectomy the increase of platelets count is due
to absence of trapped platelets in spleen.
Causes of thrombocytosis
 Primary thrombocytosis
production of platelets is independent of growth factors,the
platelets may not function normally. As in Essential
thrombocythemia.
 Reactive thrombocytosis
Secondary to some other conditions, as in IDA ,malignancy,
post splenectomy, and inflammatory disorders.
 Pseudothrombocytosis
May be seen in case of increase schistocytes (fragmented RBCs),
or WBCs fragments ( as in acute leukemia).
Qualitative platelets disorders
 Qualitative platelet disorders are characterized by
abnormalities in platelet function.
 The platelet count is usually normal but the bleeding
time is prolonged.
 Classified into two groups:
1. Congenital
2. Acquired..
 Acquired defects are more common
Congenital Platelet disorders
1. Disorder of adhesion
 Such as Bernard- Soulier Syndrome
 An autosomal recessive trait.
 Characterized by bruising and moderate to severe bleeding.
 Defect in the platelet (GPIb/IX/V) complex .
 This receptor complex mediate adhesion of platelets to the blood
vessel wall at site of injury by biding vWF and facilitate the ability
of thrombin to activate platelets.
 2. Disorder of aggregation:
 Such as Glanzmann,s thrombasthenia
 An autosomal recessive disorder in which one of two
gene coding for either the membrane glycoprotein
(GPIIb) or for (GPIIIa) is affected.
 GPIIb /IIIa – fibrinogen Aggregation
 3. Disorder of secretion
 Such as in Storage pool disorders
 They are a group of inherited conditions in which
there is a defective platelets releasing reaction due to
a lack of dense bodies and/or α granules.
 Selective deficiency in α granules also known as Grey
platelet syndrome.
Acquired Platelet Disorders
 Many conditions lead to PLTS dysfunction as :
 Uremia
 Myeloproliferative disorders(MPDs)
 Myelodysplastic disorders(MDS)
 Acute leukemia
 Dysproteinemia(as in multiple myeloma).
 Drugs ( Penicillin,dextran,aspirin …….etc)
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