XXXX : definitions and long-term outcome.

@entity1 @entity3329 @entity712 @entity1581 @entity4 @entity16 @entity12692 @entity843 @entity305 @entity120 @entity229 @entity177 @entity1897 @entity79 @entity2744 @entity7555 @entity1582 @entity715 @entity1284
The @entity7555 (coined to refer to @entity305 , @entity7555 , @entity2744 , M protein, and skin changes) remains poorly understood. Ambiguity exists over the features necessary to establish the diagnosis, treatment efficacy, and prognosis. We identified 99 @entity1 with @entity7555 . Minimal criteria were a @entity1897 and evidence of a monoclonal plasmaproliferative disorder. To distinguish POEMS from @entity16 associated with @entity1581 of undetermined significance, additional criteria were included: a @entity79 , @entity12692 , @entity7555 (or @entity3329 ), @entity2744 , @entity4 ( @entity4 , @entity843 , or effusions), and skin changes. The median age at presentation was 51 years; 63% were @entity1 . Median survival was 165 months. With the exception of fingernail clubbing (P =.03) and extravascular @entity1284 (P =.04), no presenting feature, including the number of presenting features, was predictive of survival. Response to therapy (P <.001) was predictive of survival. @entity120 , @entity177 , @entity715 events, and @entity712 were observed and appear to be part of the syndrome. In 18 @entity1 (18%), new disease manifestations developed over time. More than 50% of @entity1 had a response to radiation, and 22% to 50% had responses to @entity229 and a combination of melphalan and @entity229 , respectively. We conclude that the median survival of @entity1 with @entity7555 is 165 months, independent of the number of syndrome features, @entity79 , or plasma cells at diagnosis. Additional features of the syndrome often develop, but the complications of classic @entity1582 rarely develop.
