Adverse outcomes in primary XXXX .

@entity1 @entity2803 @entity295 @entity9058 @entity5 @entity245 @entity364 @entity204
PURPOSE: @entity9058 ( @entity9058 ) is a rare condition characterized by abnormal proliferation of macrophages. Although the mortality rate in @entity1 diagnosed with primary @entity9058 is high, little has been described about the nature of adverse events. This review evaluates unfavorable events in @entity1 with primary @entity9058 to suggest methods of improving outcomes. METHODS: Charts of @entity1 who met diagnostic criteria for primary @entity9058 at the Hospital for Sick @entity1 between January 1985 and June 2000 were retrospectively reviewed. The primary outcome measure was an adverse event, defined as death, the subsequent diagnosis of @entity5 , or @entity245 . RESULTS: Twenty @entity1 were diagnosed with primary @entity9058 . The median age at diagnosis was 6.5 months (range 1-78 months). Nineteen @entity1 received chemotherapy and two underwent matched sibling donor bone marrow transplantation. Of the 20 @entity1 , 12 (60%) died. These @entity204 were attributed to progressive @entity9058 in 4 cases and invasive infection in 8 cases. These infections consisted of disseminated @entity2803 (n = 1), @entity295 (n = 1), and invasive @entity364 (n = 6). Eight @entity1 survived. Two were subsequently diagnosed with @entity5 . Two others were found to have significant @entity245 . CONCLUSIONS: The overall mortality rate was 60% in our series of 20 @entity1 with primary @entity9058 ; 50% of @entity204 were directly attributable to invasive @entity364 . @entity245 and the diagnosis of @entity5 are important events in this cohort.
