This document discusses various platelet and coagulation disorders. It covers quantitative and qualitative platelet disorders including thrombocytopenia and Glanzman's thrombasthenia. Congenital coagulation disorders discussed are hemophilia A, hemophilia B, and von Willebrand disease. Acquired coagulation disorders include vitamin K deficiency and liver disease. Tests covered are bleeding time, prothrombin time, activated partial thromboplastin time, thrombin time, and specific factor assays. Clinical manifestations of bleeding based on location and platelet versus coagulation origin are also summarized. Hemophilia A is then discussed in more detail including inheritance, clinical severity, manifestations, and laboratory findings.

2.  Platelet disorders
 Quantitative - Thrombocytopenia
 Qualitative - Platelet function disorders – Glanzmans
 Coagulation disorders
 Congenital - Haemophilia (A, B), Von-Willebrands
 Acquired - Vitamin-K deficiency, Liver disease
 Vascular disorders –
 Scurvy
 Mixed/Consumption: DIC
Disorders of Hemostasis

3. F-XII
F-XI
F-VIII
F-IX
F-VII
F-X
F-V
F-II

4.  Screening tests:
 Bleeding Time: Platelet deficiency
 Prothrombin Time: Extrinsic, aPTT – Intrinsic
 Thrombin Time – Common path. (DIC)
 Specific tests:
 Factor assays – Hemophilia.
 Platelet function studies:
 Adhesion, Aggregation, Release tests.
 Bone Marrow study: ITP
Tests of Hemostasis:

5. Local - Vs – General
 Hematoma / Joint Bleeds- Coagulation defects
 Skin / Mucosal Bleeds – PLT
Wound / Surgical bleeding:
 Immediate - PLT
 Delayed - Coagulation
Bleeding: Clinical Features

6. Symptom Platelet Coagulation
Petechiae Yes No
Sites Skin &
Mucosa
Deep Tissue
Time Immediate Delayed
Ecchymoses
/Hematomas
Yes Yes

8.  Most common hereditary disease,
 Cause:
... Factor - VIII deficiency [ Procoagulant]
 Inheritance Pattern:
… X - linked recessive trait,
[ Sufferers - Invariably males,
Females - homozygous,
- unfavourable lionization,
… 30% no family history [ new mutations ]
HAEMOPHILIA – A:

9. Clinical severity:
 < 1% of normal activity --- severe,
 2 to 5% ” --- moderate,
 6 to 50% ” --- mild,
 Physiological activity > Type of mutation,
Clinical manifestations:
 Petechiae are absent,
 Large ecchymoses
 Prolonged bleeding
 Hemarthroses
 Hematomas
HEMOPHILIA - A :
After minor injury
or surgery
( tooth extraction )

10. Laboratory Investigations:
 Bleeding Time = Normal,
 Platelets = Normal,
 Prothrombin time = Normal,
 Clotting time
 Partial Thromboplastin Time
Haemophilia - A:
Prolonged

11. CLINICAL MANIFESTATIONS:
 Clinically indistinguishable from
Haemophilia - A
DIAGNOSIS:
 Factor Assay only;
HAEMOPHILIA - B
[ CHRISTMAS DISEASE, FACTOR - IX DEFICIENCY ]

12. Various hemorrhagic diseases and tests:
 DIC:  Prolonged aPTT, PT, D-dimer assay +
 Decreased PC
 Hemophilia  Prolonged aPTT, Normal PT, PC
 D-dimer assay (-) Decreased F VIII,
 Vit K def.  Normal aPTT, Prolonged PT, N-PC
 D-dimer assay (-) Decreased Vit K stores
 vWD  Prolonged aPTT, Normal PT, PC
 D-dimer assay (-) Decreased vWF, VIII
 ITP  Normal aPTT, PT, Decreased PC
 D-dimer assay(-) Normal vWF, VIII