Download - 3. bleeding disorders dr. sinhasan- mdzah
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
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:
Local - Vs – General Hematoma / Joint Bleeds- Coagulation defects Skin / Mucosal Bleeds – PLT
Wound / Surgical bleeding: Immediate - PLT Delayed - Coagulation
Bleeding: Clinical Features
Symptom Platelet Coagulation
Petechiae Yes No
Sites Skin & Mucosa
Deep Tissue
Time Immediate Delayed
Ecchymoses/Hematomas
Yes Yes
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:
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 )
Laboratory Investigations: Bleeding Time = Normal,
Platelets = Normal,
Prothrombin time = Normal,
Clotting time
Partial Thromboplastin Time
Haemophilia - A:
Prolonged
CLINICAL MANIFESTATIONS:
Clinically indistinguishable from
Haemophilia - A
DIAGNOSIS:
Factor Assay only;
HAEMOPHILIA - B[ CHRISTMAS DISEASE, FACTOR - IX DEFICIENCY ]
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