J 2010

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

CESARO, Simone; Judith MARSH; Gloria TRIDELLO; Alicia ROVO; Sebastien MAURY et al.

Základní údaje

Originální název

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

Autoři

CESARO, Simone; Judith MARSH; Gloria TRIDELLO; Alicia ROVO; Sebastien MAURY; Barbara MONTANTE; Tamás MASSZI; Maria Teresa VAN LINT; Boris AFANASYEV; Arturo Iriondo ATIENZA; Marc BIERINGS; Cecilia CARBONE; Michael DOUBEK; Edoardo LANINO; Mahmoud SARHAN; Antonio RISITANO; Kateřina STEINEROVÁ; Anders WAHLIN; Anna PEGORARO a Jakob PASSWEG

Vydání

Acta Haematologica, 2010, 0001-5792

Další údaje

Jazyk

angličtina

Typ výsledku

Článek v odborném periodiku

Obor

30200 3.2 Clinical medicine

Stát vydavatele

Švýcarsko

Utajení

není předmětem státního či obchodního tajemství

Impakt faktor

Impact factor: 1.316

Označené pro přenos do RIV

Ano

Kód RIV

RIV/00216224:14110/10:00051410

Organizační jednotka

Lékařská fakulta

Klíčová slova anglicky

Aplastic anemia; Autoimmune disease; Hematopoietic stem cell transplantation; Immunosuppression

Příznaky

Mezinárodní význam
Změněno: 16. 4. 2012 11:44, Mgr. Michal Petr

Anotace

V originále

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.