Internal Medicine

0521779407-22 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:23

Von Willebrand Disease 1547

differential diagnosis

■Significant bleeding history: hemophilia, other factor deficiency,

qualitative platelet defect, thrombocytopenia, collagen-vascular

defect, aspirin use

■Prolonged PTT: acquired inhibitor, antiphospholipid antibody,

intrinsic coagulation pathway factor deficiency, hemophilia

■Decreased high-molecular-weight vWf multimers: DIC, TTP, HUS

management

■Avoid aspirin.

■Head injury precautions; avoid contact sports

■Assess efficacy of DDAVP intranasal (must use Stimate brand) or

intravenous in increasing vWf activity (ristocetin cofactor) only if

multimers are normal.

■Avoid use DDAVP in type 2B vWd: risk of increased thrombosis.

■Patient education on use of Stimate at home for epistaxis, oral bleed-

ing, menorrhagia; Stimate+/−epsilon-aminocaproic acid for minor

dental work

■For major surgery, IV DDAVP 30 min prior to procedure, repeat 12

and 36 h later. For tonsillectomy and adenoidectomy, extra dose

given 5–7 days later when eschar falls off. May also require epsilon-

aminocaproic acid.

■Severe bleeding in type 3, 2N, 2M, or in type 1 vWD that does not

have a good therapeutic DDAVP trial: treat with plasma-derived

vWf/factor VIII concentrate to maintain ristocetin/vWf activity at

least 50%

■Platelet-type pseudo vWd: treat severe bleeding with platelet trans-

fusion.

■Acquired inhibitor to vWf in Wilms tumor: treat with DDAVP, cry-

oprecipitate, plasma-derived vWf/factor VIII concentrate, IVIG, or

platelets; inhibitor resolves with treatment of Wilms tumor.

specific therapy

n/a

follow-up

n/a

complications and prognosis

Pts usually do well; associated with a normal life expectancy.