Table 1. Phenotypic data of the 10... | American Society of Hematology

Table 1.

Phenotypic data of the 10 patients with von Willebrand disease

Patient	Sex	Age, y	BT, min	vWF:Ag, U/dL	RCoF, U/dL	RCoF/ vWF:Ag	vWF:CBA, U/dL	VIII:c, U/dL	RIPA, 1.3 mg/mL	RIPA, 0.5 mg/mL	Platelet count, 10⁹cells per L
A1 propositus	M	44	>20	17	6	0.35	8	25	—	—	412
A2 sister	F	41	—	18	9	0.50	12	29	nil^*	nil	294
A3 son	M	12	17	20	6	0.30	7.5	38	—	—	445
A4 nephew	M	10	>20	21	8	0.38	14	42	nil	nil	296
B1 propositus	F	31	>20	19	5	0.26	—	24	decreased	nil	221
B2 uncle	M	47	>20	25	12	0.48	—	57	nil	nil	218
C1 propositus	F	44	15.5	18	5	0.28	—	48	nil	nil	284
D1 propositus	F	33	20	20	4	0.20	—	27	nil	nil	227
E1 propositus	F	26	>20	30	18	0.60	—	58	decreased	nil	307
F1 propositus	M	54	>20	18	8	0.44	9	50	decreased	nil	254
normal range			4-8	50-150	50-150	≥0.7	50-150	50-150			150-400

Patient	Sex	Age, y	BT, min	vWF:Ag, U/dL	RCoF, U/dL	RCoF/ vWF:Ag	vWF:CBA, U/dL	VIII:c, U/dL	RIPA, 1.3 mg/mL	RIPA, 0.5 mg/mL	Platelet count, 10⁹cells per L
A1 propositus	M	44	>20	17	6	0.35	8	25	—	—	412
A2 sister	F	41	—	18	9	0.50	12	29	nil^*	nil	294
A3 son	M	12	17	20	6	0.30	7.5	38	—	—	445
A4 nephew	M	10	>20	21	8	0.38	14	42	nil	nil	296
B1 propositus	F	31	>20	19	5	0.26	—	24	decreased	nil	221
B2 uncle	M	47	>20	25	12	0.48	—	57	nil	nil	218
C1 propositus	F	44	15.5	18	5	0.28	—	48	nil	nil	284
D1 propositus	F	33	20	20	4	0.20	—	27	nil	nil	227
E1 propositus	F	26	>20	30	18	0.60	—	58	decreased	nil	307
F1 propositus	M	54	>20	18	8	0.44	9	50	decreased	nil	254
normal range			4-8	50-150	50-150	≥0.7	50-150	50-150			150-400

BT indicates bleeding time; vWF, von Willebrand factor; Ag, antigen; RCoF, ristocetin cofactor activity; CBA, collagen binding activity; VIII:c, factor VIII coagulant activity; RIPA, ristocetin-induced platelet agglutination.

Undetectable RIPA at the ristocetin concentration tested.

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