Table 1. VWD types and laboratory test... | American Society of Hematology

Table 1.

VWD types and laboratory test results

VWD type	Description	VWF:Ag	VWF activity∗	VWF activity/Ag	FVIII:C	Multimers	Other tests/results
1	Partial quantitative deficiency VWF	↓ to ↓↓	↓ to ↓↓	>0.7	Normal to ↓	Normal	N/A
1C	Increased clearance of VWF	↓ to ↓↓	↓ to ↓↓	>0.7	Normal to ↓	Normal	DDAVP trial; increased ratio of VWFpp to VWF:Ag
2A	Decreased VWF-dependent platelet adhesion with deficiency of HMWM	Normal to ↓	↓↓	<0.7	Normal to ↓	Loss of HMWM	Abnormal VWF:CB
2B	Increased affinity for platelet GPIbα	↓	↓↓	<0.7	Normal to ↓	Loss of HMWM	Abnormal VWF:CB; enhanced RIPA; genetic testing
2M	Decreased VWF-dependent platelet adhesion with normal multimers	↓	↓↓	<0.7	Normal to ↓	Normal	Normal VWF:CB
2N	Decreased binding affinity for FVIII	↓	↓↓	<0.7	Normal to ↓	Normal	Decreased VWF:FVIIIB; genetic testing
3	Virtually complete quantitative deficiency of VWF	↓↓↓	↓↓↓	N/A	↓↓↓	Absent	N/A

VWD type	Description	VWF:Ag	VWF activity∗	VWF activity/Ag	FVIII:C	Multimers	Other tests/results
1	Partial quantitative deficiency VWF	↓ to ↓↓	↓ to ↓↓	>0.7	Normal to ↓	Normal	N/A
1C	Increased clearance of VWF	↓ to ↓↓	↓ to ↓↓	>0.7	Normal to ↓	Normal	DDAVP trial; increased ratio of VWFpp to VWF:Ag
2A	Decreased VWF-dependent platelet adhesion with deficiency of HMWM	Normal to ↓	↓↓	<0.7	Normal to ↓	Loss of HMWM	Abnormal VWF:CB
2B	Increased affinity for platelet GPIbα	↓	↓↓	<0.7	Normal to ↓	Loss of HMWM	Abnormal VWF:CB; enhanced RIPA; genetic testing
2M	Decreased VWF-dependent platelet adhesion with normal multimers	↓	↓↓	<0.7	Normal to ↓	Normal	Normal VWF:CB
2N	Decreased binding affinity for FVIII	↓	↓↓	<0.7	Normal to ↓	Normal	Decreased VWF:FVIIIB; genetic testing
3	Virtually complete quantitative deficiency of VWF	↓↓↓	↓↓↓	N/A	↓↓↓	Absent	N/A

↓, slightly reduced; ↓↓, moderately reduced; ↓↓↓, significantly reduced; DDAVP, desmopressin (1-deamino-8-D-arginine vasopressin); N/A, not applicable.

∗

VWF:GPIbM (GPIb binding assay for VWF using recombinant mutated GPIb, no ristocetin) or VWF:GPIbR (GPIb binding assay for VWF using recombinant GPIb and ristocetin).

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