Von Willebrand Disease, X-Linked Form

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Retrieved

2019-09-22

Source

Omim

Trials

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Genes

VWF, P2RY12, F8, COX8A, ADAMTS13, GP1BA, F9, AK3, ABO, IL11, F11, ACTB, VWA8, ITIH1, TLR5, ITIH5, ANGPT2, POU2F3, CHRD, ALKBH1, CLEC4M, C20orf181, MUC5B, ATP6V0A2, SMPX, RABGEF1, CRISPLD2, SEC1P, PRB2, GP6, BMPER, TERF2IP, FAM20C, STXBP5, ANTXR1, ANO2, RAP1A, THPO, PDIA3, AGT, AVP, CANX, CD40, CLCA1, CSHL1, COCH, EPHA3, F2, F3, IFNA2, THBS1, RBPJ, ITGA2, ITGA2B, LRPAP1, MTHFR, CCN3, SERPINE1, PLG, POMC, SET, BOP

Drugs

Antihemophilic factor / Von Willebrand factor complex (human) ( ALPHANATE, HUMATE -P ), Desmopressine acetate ( DESMOPRESSIN ACETATE AVENTIS BEHRING L.L.C., OCTIM, MINIRIN ), Recombinant human von Willebrand factor ( VONVENDI )

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Holmberg and Nilsson (1973) used a monospecific precipitating rabbit antiserum against human antihemophilic factor-related protein to study 77 patients with von Willebrand disease (defined by low factor VIII, prolonged bleeding time and decreased platelet adhesiveness). They found two groups of patients. The larger group (57 patients) corresponded to classic von Willebrand disease and had low factor VIII protein. The other 20 patients had normal amounts of protein. Their disorder may be X-linked dominant. Infusion of human antihemophilic factor containing fraction I-O did not produce the delayed increase in antihemophilic factor characteristic of the first group. Various forms were suggested also by Koutts et al. (1975).