OBJECTIVES: To evaluate the occurrence of @entity792 in @entity17454 ( @entity17454 ) and to determine risk factors and the prognosis of such complications with the aim of improving the definition of treatment options. METHODS: We reviewed the records of 55 @entity1 with @entity17454 evaluated at Necker-Enfants Malades Hospital (Paris) from 1980 to 2000. RESULTS: Forty @entity1 (72%) had at least 1 autoimmune or inflammatory complication. @entity5506 was detected in 20 cases (36%); in all cases, onset occurred before the age of 5 years. Other complications included @entity1398 (25%), @entity625 (29%), @entity1333 (22%), @entity12513 (7%), @entity29 (9%), and @entity8 (3%). The median survival of the entire population was 14.5 years. Two @entity792 and 1 biological factor were predictive of a poor prognosis in this population: @entity5506 , severe @entity882 recurring after splenectomy, and high serum immunoglobulin M (IgM) levels before splenectomy. @entity5506 was significantly more observed in @entity1 with high serum IgM level. CONCLUSIONS: High serum IgM concentration before splenectomy was identified as a risk factor for @entity5506 ; however, it must be confirmed. @entity5506 and severe @entity882 recurring after splenectomy were 2 indicators of a poor prognosis. Those results suggest that @entity1 with @entity17454 and IgM levels more than mean + 2 standard deviations before splenectomy should be placed under strict surveillance. Furthermore, severe @entity792 should lead, as early as possible, to hematopoietic stem cell transplantation using the best available donor.

Autoimmunity in @entity17454 : risk factors, clinical features, and outcome in a single-center cohort of 55 XXXX .

@entity1 :: ('9606', 'Species') :: ['patients'] @entity8 :: ('MESH:D007674', 'Disease') :: ['renal disease'] @entity12513 :: ('MESH:D020293', 'Disease') :: ['cerebral vasculitis'] @entity625 :: ('MESH:D001168', 'Disease') :: ['arthritis'] @entity882 :: ('MESH:D013921', 'Disease') :: ['thrombocytopenia'] @entity1333 :: ('MESH:D014657', 'Disease') :: ['skin vasculitis'] @entity17454 :: ('MESH:D014923', 'Disease') :: ['WAS', 'Wiskott-Aldrich syndrome'] @entity29 :: ('MESH:D015212', 'Disease') :: ['inflammatory bowel disease'] @entity5506 :: ('MESH:D000744', 'Disease') :: ['Autoimmune hemolytic anemia', 'autoimmune hemolytic anemia'] @entity1398 :: ('MESH:D009503', 'Disease') :: ['neutropenia'] @entity792 :: ('MESH:D001327', 'Disease') :: ['autoimmune and inflammatory complications', 'autoimmune complications']
@entity1 :: (9606,Species) :: ['patients']
