TY - JOUR
T1 - Platelet-type von Willebrand's disease; Characterization of a new bleeding disorder
AU - Miller, J. L.
AU - Castella, A.
PY - 1982
Y1 - 1982
N2 - An autosomally transmitted bleeding diathesis sharing some, but not all, features previously described in von Willebrand's disease (vWd) was studied in five patients representing three generations of a single family. Bleeding times in the upper normal range in conjunction with low-normal platelet counts, normal factor VIII coagulant activity and VIII-related antigen, decreased VIII-ristocetin cofactor activity, selective decrease of the higher molecule weight factor VIII/von Willebrand factor (VIII/vWf) multimers, and increased ristocetin-induced platelet agglutination at low ristocetin concentrations were characteristic. Binding of patient VIII/vWF to washed normal platelets was within normal limits, whereas binding of normal VIII/vWF to patient platelets was significantly increased (p < 0.001 at 0.6 mg/ml ristocetin). This disorder accordingly appears to involve an intrinsic platelet abnormality affecting platelet-VIII/vWF interactions. It is proposed that this concept of vWD be broadened to include patients with this abnormality, which may appropriately be called 'Platelet-type von Willebrand's disease'.
AB - An autosomally transmitted bleeding diathesis sharing some, but not all, features previously described in von Willebrand's disease (vWd) was studied in five patients representing three generations of a single family. Bleeding times in the upper normal range in conjunction with low-normal platelet counts, normal factor VIII coagulant activity and VIII-related antigen, decreased VIII-ristocetin cofactor activity, selective decrease of the higher molecule weight factor VIII/von Willebrand factor (VIII/vWf) multimers, and increased ristocetin-induced platelet agglutination at low ristocetin concentrations were characteristic. Binding of patient VIII/vWF to washed normal platelets was within normal limits, whereas binding of normal VIII/vWF to patient platelets was significantly increased (p < 0.001 at 0.6 mg/ml ristocetin). This disorder accordingly appears to involve an intrinsic platelet abnormality affecting platelet-VIII/vWF interactions. It is proposed that this concept of vWD be broadened to include patients with this abnormality, which may appropriately be called 'Platelet-type von Willebrand's disease'.
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U2 - 10.1182/blood.v60.3.790.790
DO - 10.1182/blood.v60.3.790.790
M3 - Article
C2 - 6286015
AN - SCOPUS:0019998246
SN - 0006-4971
VL - 60
SP - 790
EP - 794
JO - Blood
JF - Blood
IS - 3
ER -