Table 1. Demographics and laboratory... | American Society of Hematology

Table 1.

Demographics and laboratory values of patients with type 2B VWD on day 1

	Units	Patient ID
	Units	1	2	3	4	5
Mutation		R1308C	R1306W	R1306W	V1316M	W1313C
Prophylaxis		rVWF	No	No	rVWF	No
Sex		Female	Male	Male	Male	Female
Age	y	64	61	24	72	61
Weight	kg	94	100	100	91	77
Height	cm	163	177	180	175	158
Platelets	×10E9/L	41	50	120	60	167
VWF:Ag	%	106	64	32	69	40
FVIII	%	91	67	44	84	64
VWF:GpIbM	%	42	25	19	18	3
VWF:RCo	%	30	31	17	17	9
VWF:CB	%	72	50	19	52	15
VWF:RCo/VWF:Ag ratio		0.28	0.48	0.53	0.25	0.23
VWFpp/VWF:Ag ratio		21	25	47	18	27
VWFpp	mIU/mL	2229	1614	1506	1252	1095
CADP-CT	s	301	301	301	301	301
Ristocetin induced aggregation	U	55	34	31	120	29
VWF intermediate molecular weight multimers		Reduced	Reduced	Reduced	Reduced	Reduced
VWF high molecular weight multimers		Reduced	Reduced	Reduced	Reduced	Reduced
Bleeding score		30	20	16	19	21

	Units	Patient ID
	Units	1	2	3	4	5
Mutation		R1308C	R1306W	R1306W	V1316M	W1313C
Prophylaxis		rVWF	No	No	rVWF	No
Sex		Female	Male	Male	Male	Female
Age	y	64	61	24	72	61
Weight	kg	94	100	100	91	77
Height	cm	163	177	180	175	158
Platelets	×10E9/L	41	50	120	60	167
VWF:Ag	%	106	64	32	69	40
FVIII	%	91	67	44	84	64
VWF:GpIbM	%	42	25	19	18	3
VWF:RCo	%	30	31	17	17	9
VWF:CB	%	72	50	19	52	15
VWF:RCo/VWF:Ag ratio		0.28	0.48	0.53	0.25	0.23
VWFpp/VWF:Ag ratio		21	25	47	18	27
VWFpp	mIU/mL	2229	1614	1506	1252	1095
CADP-CT	s	301	301	301	301	301
Ristocetin induced aggregation	U	55	34	31	120	29
VWF intermediate molecular weight multimers		Reduced	Reduced	Reduced	Reduced	Reduced
VWF high molecular weight multimers		Reduced	Reduced	Reduced	Reduced	Reduced
Bleeding score		30	20	16	19	21

Abnormal values are highlighted in bold. Mutations are indicated by amino acid substitutions (https://www.omim.org/entry/613160#73, www.VWF.group.shef.ac.uk; last accessed 18 December 2021). Patients 2 and 3 are father and son; patient 5 has an additional amino acid polymorphism p.(V1565L) in the A2 domain.

CADP-CT, collagen adenosine diphosphate-closure time (s); NA, not assessed; VWF:Ag, von Willebrand factor antigen (%); FVIII, factor VIII clotting activity (%); VWF:CB, VWF collagen binding activity (%); VWF:GpIbM, VWF glycoprotein Ib mutation activity (%); VWFpp, VWF propeptide (mIU/mL); VWF:RCo, VWF ristocetin cofactor activity (%).

View Large

This Feature Is Available To Subscribers Only