Retrospective Survey on the Prevalence and Outcome of Prior Autoimmune Diseases in Patients with Aplastic Anemia Reported to the Registry of the European Group for Blood and Marrow TransplantationCesaro S.a · Marsh J.b · Tridello G.a · Rovò A.c · Maury S.d · Montante B.e · Masszi T.f · Van Lint M.T.g · Afanasyev B.h · Iriondo Atienza A.i · Bierings M.j · Carbone C.k · Doubek M.l · Lanino E.m · Sarhan M.n · Risitano A.o · Steinerova K.p · Wahlin A.q · Pegoraro A.a · Passweg J.r
aPediatric Hematology Oncology, Department of Pediatrics, University of Padua, Padua, Italy; bDepartment of Haematological Medicine, King’s College Hospital, London, UK; cDepartment of Hematology, University Hospital, Basel, Switzerland; dDepartment of Hematology, Henri Mondor Hospital, Créteil, France; eHematology and SCT Unit, S. Camillo Hospital, Rome, Italy; fDepartment of Hematology and Stem Cell Transplantation, St. István and St. Laszlo Hospital, Budapest, Hungary; gDepartment of Hematology II, Ospedale San Martino, Genoa, Italy; hDepartment of Hematology and Transplantology, St. Petersburg State Medical Pavlov University, St. Petersburg, Russia; iDepartment of Hematology, Marqués de Valdecilla University Hospital, Santander, Spain; jDepartment of Hematology, University Hospital for Children (WKZ), Utrecht, The Netherlands; kDepartment of Hematology, Spedali Civili, Brescia, Italy; lDepartment of Internal Medicine, Hematology and Oncology, University Hospital, Brno, Czech Republic; mDepartment of Pediatric Hematology Oncology, G. Gaslini Institute, Genoa, Italy; nKing Hussein Cancer Centre, Amman, Jordan; oDivision of Hematology, ‘Federico II‘ Medical School, University of Naples, Naples, Italy; pDepartment of Hematology/Oncology, Charles University Hospital, Pilsen, Czech Republic; qDepartment of Hematology, Umea University Hospital, Umeå, Sweden; rDepartment of Internal Medicine, University Hospital, Geneva, Switzerland
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Background: Aplastic anemia (AA) is rarely described after a diagnosis of autoimmune disease (aID). Aims: To assess the prevalence of prior aID in patients with AA recorded in the registry of the European Group for Blood and Marrow Transplantation (EBMT) and to evaluate treatment and outcome. Methods: 1,251 AA patients from 18 EBMT centers were assessed. Results: Fifty patients (4%) were eligible: 22 males and 28 females with a median age of 46 years at the diagnosis of aID and of 51 years at the diagnosis of AA. Information on the treatment of AA was available in 49 patients: 38 received only immunosuppressive therapy (IST), 8 patients underwent hematopoietic stem cell transplantation (HSCT) – 6 as first-line therapy and 2 after failure of IST – whilst 3 patients had a spontaneous recovery. After a median follow-up of 3.19 years, 32 patients were alive, including 7 of the 8 patients who underwent HSCT. Only 6 of 32 patients who were alive at the last follow-up were receiving IST for AA. Conclusions: Most cases of AA following aID benefitted from IST or HSCT if a matched donor was available. Further prospective investigation is needed to assess the effects of IST on the outcome of underlying aID.
© 2010 S. Karger AG, Basel
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