Bleeding Disorders : Therapy

a. Thrombocytopenia caused by drugs
- Discontinue use of possible offending agents, except recovery in 7-10 days. Platelet transfusions may be needed if platelet count on < style="font-weight: bold;">b. Heparin-Induced Thrombocytopenia
- Discontinue heparin promptly.
- A direct thrombin inhibitor such as lepirudin (0,4-mg/kg bolus, 0,15-mg/kg per hour infusion, PTT target 1,5-2,5 x baseline) should be used for treatment of thromboses.
- Do not use low-molecular-weight heparin (LWMH), as antibodies often cross react.

c. Chronic ITP
- Prednison, initially 1-2 mg/kg per day, then slow taper to keep the platelet count > 60.000/µL.
- IV immunoglobulin (2 g/kg in devided doses oveeer 2-5 days) to block phagocytic destruction may be useful.
- Rituximab is effective in patients refractory to glucocorticoids.
- Splenectomy, danazol (androgen) or other agents (e.g., vincristine, cyclophosphamide, fludarabine) are indicated for refractory patients or those requiring >5-10 mg prednisone daily.

d. DIC
- Control of underlying disease most important, platelets, fresh frozen plasma (FFP) to correct clotting parameters.
- Heparin may be beneficial in patients with acute promyelocytic leukemia.

e. TTP
- Plasmapheresis and FFP infusions (plasma exchange), possibly IV IgG, recovery in two-thirds of cases.
- Plasmapheresis removes inhibitors of the vWF cleavage enzyme (ADAMTS13), and FFP replaces the enzyme.

f. Disorders of Platelet Function
- Remove or reverse underying cause
- Dialysis and/or cryoprecipitate infusions (10 bags/24h) may be helpful for platelet dysfunction associated with uremia

g. Hemostatic Disorders
- Withdraw offending drugs, replace vitamin C, plasmapheresis, and plasma infusion for TTP.

h. Hemophilia A
- Factor VIII replacement for bleeding or befor surgical procedure, degree and duration of replacement depends on severity of bleeding.
- Give factor VIII (e.g. Recombinate) to obtain a 15% (for mild bleeding) to 50% (for severe bleeding) factor VIII level.
- The duration should range from a single dose of factor VIII to therapy bid for up to 2 weeks. Dose is calculated as follows

Factor VIII dose + (target level-baseline level) x weight (kg) x 0,5 unit/kg.

i. Hemophilia B
- Recombinant factor IX (e.g Benefix), FFP or factor IX concentrates (e.g., Proplex, Konyne).
- Because of the longer half-life, once daily treatment is sufficient.
- Dose is calculated as follows :

Factor IX dose = (Target level-baseline level) x weight (kg) x1 unit/kg

j. Von Willebrand Disease
- Desmopresin (1-deamino-8-D arginine vasopressin increases release of vWF from endothelial stores in type 1 vWD.
- It is given IV (0,3 µg/kg) or by nasal spray (2 squirts of 1,5 mg/mL luid in each nostril).
- For types 2A, 2M, and 3, cryoprecipitate (plasma product rich in factor VIII) or factor VIII concentrate (Humate-P, Koate HS) is used up to 10 bags bid 72 h, depending on severity of bleeding.


k. Vitamin K deficiency
- Vitamin K, 10 mg SC or slow IV

l. Liver Disease
- Fresh-frozen plasma


17th Edition Harrison’s Manual of Medicine, 2009 Chap. 68. Bleeding and Thrombotic Disorders p. 332
For more detailed, see
Konkle BA : Bleeding and Thrombosis, Chap 59, p.363
Konkle BA : Disorders of platelets and Vessel Wall, chap 109, p. 718
Arruda V, High KA : Coagulation Disorders, chap 110, p.725
Weititz JI : Antiplatelet, Anticoagulant, and Fibrionolytic Drugs, chap 112, p. 735, in HPIM-17