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 Transplantation

• Authors: CESARO Simone; MARSH Judith; TRIDELLO Gloria; ROVO Alicia; MAURY Sebastien; MONTANTE Barbara; MASSZI Tamás; VAN LINT Maria Teresa; AFANASYEV Boris; ATIENZA Arturo Iriondo; BIERINGS Marc; CARBONE Cecilia; DOUBEK Michael; LANINO Edoardo; SARHAN Mahmoud; RISITANO Antonio; STEINEROVÁ Kateřina; WAHLIN Anders; PEGORARO Anna; PASSWEG Jakob
• Year of publication: 2010
• Type: Article in Periodical
• Magazine / Source: Acta Haematologica
• MU Faculty or unit: Faculty of Medicine
• Doi: http://dx.doi.org/10.1159/000313783
• Field: Oncology and hematology
• Keywords: Aplastic anemia; Autoimmune disease; Hematopoietic stem cell transplantation; Immunosuppression
• Description: 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.