1. The document discusses common screening tests for bleeding disorders including bleeding time, platelet count, prothrombin time, and partial thromboplastin time. 2. It also lists and describes common bleeding disorders such as immune thrombocytopenic purpura, hemophilia A, hemophilia B, von Willebrand disease, and disseminated intravascular coagulation. 3. The pathogenesis, clinical features, and treatment of immune thrombocytopenic purpura are explained in detail, noting it is caused by autoantibodies destroying platelets and treated commonly with corticosteroids or splenectomy.

1. HEMORRHAGIC DIATHESIS I
(BLEEDING DISORDERS)
Dr. S. Vaidya,
Dept. of Pathology

2. OBJECTIVES
1. list of common basic screening tests in bleeding
disorders & its significance.
2. list of common bleeding disorders
3. aetiopathogenesis and laboratory diagnosis of
immune thrombocytopenic purpura (ITP).

3. HEMORRHAGIC DIATHESIS (BLEEDING
DISORDERS)
• are a group of disorders characterized clinically by
abnormal bleeding, which can be either spontaneous
or become evident after some inciting event
(trauma, surgery)

4. NORMAL HEMOSTASIS
• normal hemostasis is a consequence of tightly regulated
processes that maintain blood in a fluid state in normal
vessels, yet also permit the rapid formation of a
hemostatic clot at the site of a vascular injury
• normal hemostasis depends upon normal function of:
1. endothelium (blood vessel)
2. platelets
3. coagulation factors

5. SCREENING TESTS FOR HAEMOSTASIS
1. Bleeding time (BT):
• measures the time taken for a standardized skin
puncture to stop bleeding.
• normal range: 2 - 9 minutes
• prolonged BT indicates a defect in platelet numbers
or function.

6. 2. Platelet count:
• are obtained using anticoagulated blood
a. manually
b. electronic particle counter
• normal range: 150 to 450x103/µl.
• associated disorders :
a. thrombocytopenia
b. thrombocytosis

8. 3. Prothrombin time (PT):
• this assay tests the extrinsic & common coagulation
pathways.
• normal range: 10 - 14 seconds.
⮚ prolonged PT: due to deficiency or dysfunction of
factors V, VII, factor X, prothrombin (II), or fibrinogen
(I).

9. 4. Partial thromboplastin time(PTT):
• this assay tests the intrinsic and common
coagulation pathways.
• normal range: 30 - 40 seconds.
⮚ Prolonged PTT: due to deficiency or dysfunction of
factors V, VIII, IX, X, XI, or XII, prothrombin (II), or
fibrinogen (I).

10. • other specialized tests available are:
1. levels of specific clotting factors
2. fibrinogen
3. fibrin split products
4. presence of circulating anticoagulants.
5. platelet adhesion tests
6. platelet aggregation tests

11. BLEEDING DISORDERS
• the causes of bleeding disorders maybe broadly
divided into:
1. bleeding disorders due to vascular abnormalities
2. bleeding disorders due to platelet abnormalities
3. disorders of coagulation factors
4. combination of all of the above (Disseminated
Intravascular Coagulation / DIC)

12. LIST OF COMMON BLEEDING DISORDERS
1. immune thrombocytopenic purpura (ITP)
2. hemophilia A
3. hemophilia B
4. von Willebrand disease
5. disseminated intravascular coagulation (DIC)

13. IMMUNE THROMBOCYTOPENIC PURPURA
(ITP)
• immune thrombocytopenic purpura (ITP) is
characterized by immunologic destruction of
platelets and normal or increased megakaryocytes in
the bone marrow.

16. • on the basis of duration of illness, ITP is classified
into:
1. acute ITP
1. chronic ITP

17. CHRONIC ITP
• occurs more commonly in adults, particularly in
women of child-bearing age.
• age: 40,
• M:F ratio = 1:3
• it can be:
1. primary: absence of any known risk factors
2. secondary: occur in variety of predisposing
conditions and exposures

18. PATHOGENESIS
• also caused by autoantibodies to platelets.
⮚ autoantibodies, most often directed against platelet
membrane glycoproteins IIb-IIIa or Ib-IX

19. CLINICAL FEATURES
1. petechial haemorrhages
2. easy bruising
3. mucosal bleeding: menorrhagia in women, nasal
bleeding, bleeding from gum, melaena and
haematuria.
4. intracranial haemorrhage (rare)
5. hepatomegaly
6. splenomegaly (uncommon)

20. ACUTE ITP
• self-limited disorder, seen most frequently in children
following recovery from viral infection or upper
respiratory infection.
⮚ caused by antiplatelet antibodies
• onset of acute ITP is sudden & severe thrombocytopenia
but recovery occurs within a few weeks to 6 months.
⮚ in about 20% of children have a childhood form of
chronic ITP that follows a course similar to the adult
disease.

21. LABORATORY FINDINGS
1. platelet count: markedly reduced.
2. blood film: shows occasional platelets.
3. bone marrow: shows increased number of
megakaryocytes.
4. anti-platelet IgG antibody: can be demostrated in
the serum of patients.
5. platelet survival studies: markedly reduced platelet
lifespan (normal 7-10 days).

22. TREATMENT
• spontaneous recovery in 90% cases of acute ITP.
• corticosteroid therapy
• splenectomy:
⮚ thrombocytopenia is improved by splenectomy,
indicating that the spleen is the major site of
removal of platelets.

23. THANK YOU