The 5q-syndrome and autoimmune phenomena: Report of three cases

The 5q-syndrome and autoimmune phenomena: Report of three cases

Author Pinheiro, R. F. Google Scholar
Silva, MRR Google Scholar
Chauffaille, MDF Google Scholar
Institution Universidade Federal de São Paulo (UNIFESP)
Abstract Myelodysplastic syndrome is a clonal hematopoietic stem cell disorder characterized by ineffective hematopoiesis, peripheral cytopenias and an additional risk to evolve to acute leukemia in up to 30% of the cases. Autoimmune manifestations as vasculitis, pyoderma gangrenosum, hemolytic anemia, immune thrombocytopenia, rheumatoid arthritis as well as positive anti-nuclear factor and rheumatoid factor have been reported in 13-30% of MDS patients. the aim of this report is to present three patients with 5q- syndrome who presented different autoimmune, serological and clinical phenomena and review the literature. Patient I showed a focal and segmental glomerulosclerosis (FSGE) in the course of a MDS. Renal involvement in MDS as autoimmune phenomenon is rare and few reports have documented different forms of glomerular diseases in adults with MDS. Patients 2 and 3 showed a rheumatoid factor of 1/140 and the direct Coomb's test positive (3+), respectively, but without evidence of clinical autoimmune manifestation.In conclusion, patients with the 5q- syndrome experience a relative benign disease course extending over several years. We believe that careful follow-up of patients with autoimmune manifestations as here reported is important to detect any unexpected outcome. (C) 2005 Elsevier B.V. All rights reserved.
Keywords myelodysplatic syndrome
5q
syndrome
autoimmune manifestations
Language English
Date 2006-04-01
Published in Leukemia Research. Oxford: Pergamon-Elsevier B.V., v. 30, n. 4, p. 507-510, 2006.
ISSN 0145-2126 (Sherpa/Romeo, impact factor)
Publisher Elsevier B.V.
Extent 507-510
Origin http://dx.doi.org/10.1016/j.leukres.2005.08.025
Access rights Closed access
Type Article
Web of Science ID WOS:000236086200020
URI http://repositorio.unifesp.br/handle/11600/28819

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