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Von Willebrand Disease - Type 2b

View larger version (52K): [in this window] [in a new window]   Figure 1. Platelet aggre-gation pattern of normal(red) and patient(blue) plasmas with 1.5mg/dL Ristocetin. Patient had a normal aPTT, factor VIII activity of 77%, VWF antigen of 61%, Ristocetin co-factor activity of <12.5%, and a VWF multimer pattern showing absence of high molecular weight multimers. Differential diagnosis was VWD type 2b versus platelet-type (pseudo) VWD.

View larger version (26K): [in this window] [in a new window]   Figure 2. Control platelet-rich plasma showed a minimal aggregatory response to low-dose Ristocetin (0.5mg/dL).

View larger version (33K): [in this window] [in a new window]   Figure 3. Patient's platelet-rich plasma aggregation response to low-dose Ristocetin (0.5mg/dL) showing complete aggregation.

View larger version (34K): [in this window] [in a new window]   Figure 4. Control platelets were mixed in a 1:1 ratio with normal plasma (blue) and cryoprecipitate (red), and the mixtures aggregated with low-dose Ristocetin (0.5mg/dL).

View larger version (38K): [in this window] [in a new window]   Figure 5. A 1:1 mixture of control platelets and the patient's plasma was aggregated with low-dose Ristocetin (0.5mg/dL). The aggregation response confirmed that the loss of high molecular weight multimers was due to VWD type 2b rather than pseudo-von Willebrand disease. If the abnormality had been due to platelet-type (pseudo) VWD, the patient's plasma would not have caused normal platelets to respond to low-dose Ristocetin.

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This Article Figures Only Services Similar articles in this journal Alert me to new content in the Image Bank Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Lazarchick, J. Search for Related Content PubMed Articles by Lazarchick, J. Related Collections Coagulation/Fibrinolytic Factor Deficiencies Related ASH-SAP Chapter Related Image Bank Image Sets Related ASH Education Book Article Social Bookmarking                   What's this?