Misdiagnosis of XXXX in childhood.

@entity1 @entity4343 @entity882 @entity1423 @entity230 @entity3039
PURPOSE: @entity1 ultimately diagnosed with nonimmune @entity882 are often referred to pediatric hematology clinics with a provisional diagnosis of @entity3039 ( @entity3039 ). The authors' aim was to establish in these @entity1 the features characterizing the mechanism of @entity882 . @entity1 AND METHODS: The authors performed a retrospective review of the case records of seven @entity1 (three @entity1 and four @entity1 , aged 5 months to 7 years) with misdiagnosed chronic @entity3039 referred to a single pediatric hematology center between 1990 and 2000. RESULTS: In the seven @entity1 , the suspected diagnosis on referral was @entity3039 and the final diagnosis was @entity882 . @entity1423 and/or leukocyte morphology were present in all of them. Other features suggestive of @entity882 included a history of @entity882 (2/7), failure of @entity230 and/or intravenous immunoglobulins to raise the platelet count to normal levels (5/7), and moderate increase of @entity4343 -111 platelet turnover in the two @entity1 tested. Platelet-associated IgG (PaIgG) was above the normal threshold in the four @entity1 tested; the direct monoclonal antibody immobilization of platelet antigens (MAIPA) test was negative in the four @entity1 tested and the serum test was positive in two @entity1 . Bone marrow examination revealed either a normal (4/7) or an elevated (3/7) number of megakaryocytes. CONCLUSIONS: Family history and blood cell morphology analysis in experienced hands are the first steps in discriminating @entity3039 from @entity882 in @entity1 with isolated @entity882 . In contrast, bone marrow examination and search for specific autoantibodies using the MAIPA test are of little help. An isotopic platelet life span study, when available, should be performed before considering splenectomy to exclude the diagnosis of @entity882 , especially when @entity230 and/or IgG IV administration failed to raise the platelet count.
