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Title: The 5q-syndrome and autoimmune phenomena: Report of three cases
Authors: Pinheiro, R. F.
Silva, MRR
Chauffaille, MDF
Universidade Federal de São Paulo (UNIFESP)
Keywords: myelodysplatic syndrome
autoimmune manifestations
Issue Date: 1-Apr-2006
Publisher: Elsevier B.V.
Citation: Leukemia Research. Oxford: Pergamon-Elsevier B.V., v. 30, n. 4, p. 507-510, 2006.
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.
ISSN: 0145-2126
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