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.
BACKGROUND
Aplastic anemia (AA) is rarely described after a diagnosis of autoimmune disease (aID).


OBJECTIVE
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.
Cesaro
Simone
S
Pediatric Hematology Oncology, Department of Pediatrics, University of Padua, Padua, Italy. simone.cesaro@azosp.vr.it
Marsh
Judith
J
Tridello
Gloria
G
Rovò
Alicia
A
Maury
Sebastien
S
Montante
Barbara
B
Masszi
Tamás
T
Van Lint
Maria Teresa
MT
Afanasyev
Boris
B
Iriondo Atienza
Arturo
A
Bierings
Marc
M
Carbone
Cecilia
C
Doubek
Michael
M
Lanino
Edoardo
E
Sarhan
Mahmoud
M
Risitano
Antonio
A
Steinerova
Katerina
K
Wahlin
Anders
A
Pegoraro
Anna
A
Passweg
Jakob
J
D000293:Adolescent; D000328:Adult; D000368:Aged; D000741:Anemia, Aplastic / Q000453:epidemiology* / Q000209:etiology / Q000628:therapy; D001327:Autoimmune Diseases / Q000150:complications / Q000453:epidemiology* / Q000628:therapy; D016026:Bone Marrow Transplantation; D002648:Child; D003625:Data Collection; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D012042:Registries; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome; D055815:Young Adult
D007166:Immunosuppressive Agents