TY - JOUR T1 - Autoimmune hematological diseases after allogeneic hematopoietic stem cell transplantation in children: an Italian multicenter experience. JF - Biol Blood Marrow Transplant Y1 - 2014 A1 - Faraci, Maura A1 - Zecca, Marco A1 - Pillon, Marta A1 - Rovelli, Attilio A1 - Menconi, Maria Cristina A1 - Ripaldi, Mimmo A1 - Fagioli, Franca A1 - Rabusin, Marco A1 - Ziino, Ottavio A1 - Lanino, Edoardo A1 - Locatelli, Franco A1 - Daikeler, Thomas A1 - Prete, Arcangelo KW - Child KW - Child, Preschool KW - Female KW - Hematologic Diseases KW - Hematopoietic Stem Cell Transplantation KW - Humans KW - Italy KW - Male KW - Remission Induction KW - Risk Factors KW - Transplantation Conditioning AB -

Autoimmune hematological diseases (AHDs) may occur after allogeneic hematopoietic stem cell transplantation (HSCT), but reports on these complications in large cohorts of pediatric patients are lacking. Between 1998 and 2011, 1574 consecutive children underwent allogeneic HSCT in 9 Italian centers. Thirty-three children (2.1%) developed AHDs: 15 autoimmune hemolytic anemia (45%), 10 immune thrombocytopenia (30%), 5 Evans' syndrome (15%), 2 pure red cell aplasia (6%), and 1 immune neutropenia (3%). The 10-year cumulative incidence of AHDs was 2.5% (95% confidence interval, 1.7 to 3.6). In a multivariate analysis, the use of alternative donor and nonmalignant disease was statistically associated with AHDs. Most patients with AHDs (64%) did not respond to steroids. Sustained complete remission was achieved in 87% of cases with the anti-CD20 monoclonal antibody (rituximab). Four patients (9%) (1 autoimmune hemolytic anemia, 1 Evans' syndrome, 2 immune thrombocytopenia) died at a median of 87 days after AHD diagnosis as a direct or indirect consequence of their disorder. Our data suggest that AHDs are a relatively rare complication occurring after HSCT that usually respond to treatment with rituximab.

VL - 20 IS - 2 U1 - http://www.ncbi.nlm.nih.gov/pubmed/24274983?dopt=Abstract ER -