Case Reports in Hematology / 2015 / Article / Tab 1

Case Report

Successful Aortic Aneurysm Repair in a Woman with Severe von Willebrand (Type 3) Disease

Table 1

Classification of VWD (adapted from Laffan et al. [3]).

Type Nature of VWF defectPhenotypic changesMode of inheritance

1Partial quantitative deficiency (i) Includes rapid VWF clearance (e.g., VWF Vicenza) mutations
(ii) Requires VWF:RCo/VWF:Ag ratio >0.6
(i) Predominately autosomal dominant inheritance when VWF <0.3 IU/dL
(ii) VWF mutations with levels >0.3 IU/dL show variable penetrance
2Qualitative defects
2ADecreased VWF-dependent platelet adhesion with selective deficiency of HMWMVWF:RCo/VWF:Ag ratio <0.6(i) Mostly autosomal dominant
2BIncreased affinity for platelet GPIb(i) VWF:RCo/VWF:Ag ratio <0.6
(ii) Typically associated with thrombocytopenia
(iii) Should be distinguished from PT-VWD, by further testing by platelet agglutination tests or genetic testing
(iv) Cases with normal VWF multimer and platelet count have been described
(i) Autosomal dominant
2MDecreased VWF-dependent platelet adhesion without selective deficiency of HMWM(i) This also includes defects of VWF collagen binding (i) Autosomal dominant
2NMarkedly decreased binding affinity for FVIII(i) VWF:RCo/VWF:Ag ratio <0.7
(ii) Should be distinguished from mild haemophilia A
(i) VWF:FVIII binding defects are more commonly due to compound heterozygote with a VWF null allele rather than the classical homozygous state
3Virtually complete deficiency (i) Equivalent to <0.03 IU/dL in most assays
(ii) Bleeding symptoms in up to 48% of obligate carriers
(i) Autosomal recessive, frequent null VWF alleles

HMWM = high molecular weight multimers, PT-VWD = platelet type VWD.

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