Acquired XXXX : a single institution experience.

@entity1 @entity7062 @entity548 @entity706 @entity1415 @entity30 @entity1072 @entity3459 @entity8325 @entity1503 @entity2881 @entity360 @entity2880
Acquired @entity2880 (AVWS) is a rare @entity3459 distinguished from @entity2880 by age at presentation and absence of personal and family history of @entity3459 . We report on 22 @entity1 with AVWS seen over 25 years. Mean age at diagnosis was 61.3 years (range 38-86 years); most @entity1 had a spontaneous or a post-operative @entity548 at presentation. @entity360 and @entity1415 were the most common spontaneous symptoms. Bleeding time was prolonged in most @entity1 , associated with marked reductions in plasma @entity2880 factor antigen and @entity8325 cofactor activity. Plasma @entity2881 multimer distribution was normal (type 1 pattern) in 5 @entity1 , indeterminate (no multimers detectable) in 6 @entity1 (type 3 pattern), and abnormal (decreased higher-molecular-weight multimers, type 2 pattern) in 11 @entity1 . None of 17 @entity1 tested had an inhibitor of @entity8325 cofactor activity. An underlying @entity1503 was found in 18 @entity1 , and 1 @entity1 had @entity30 . @entity7062 was effective in only half the @entity1 so treated, but all @entity1 responded to treatment with @entity2881 -containing concentrates. Resolution of AVWS occurred with therapy of @entity706 (1 @entity1 ) and @entity1072 (1 @entity1 ). Sixteen @entity1 were alive at last follow-up; no deaths were related to @entity548 . AVWS may be more prevalent than has been appreciated; we estimate up to 0.04%. Awareness of the existence of AVWS is essential for diagnosis and appropriate management. Therapy of associated diseases may improve the @entity3459 .
