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

被引:6
作者
Pinheiro, RF
Silva, MRR
Chauffaille, MDF
机构
[1] Univ Fed Sao Paulo, EPM, Disciplina Hematol & Hemoterapia, BR-04023900 Sao Paulo, Brazil
[2] Univ Fed Sao Paulo, EPM, Disciplina Anat Patol, Sao Paulo, Brazil
基金
巴西圣保罗研究基金会;
关键词
myelodysplatic syndrome; 5q; syndrome; autoimmune manifestations;
D O I
10.1016/j.leukres.2005.08.025
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
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 Ltd. All rights reserved.
引用
收藏
页码:507 / 510
页数:4
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