Utilize este identificador para referenciar este registo: http://hdl.handle.net/10400.16/515
Título: CD8 (+)/V beta 5.1(+) large granular lymphocyte leukemia associated with autoimmune cytopenias, rheumatoid arthritis and vascular mammary skin lesions: successful response to 2‐deoxycoformycin.
Autor: GRANJO, E.
LIMA, M.
CORREIA, T.
LISBOA, C.
MAGALHAES, C
CUNHA, N.
TEIXEIRA, M.A.
QUEIROS, M.L.
Data: Jun-2002
Editora: Wiley-Blackwell
Resumo: Hematol Oncol. 2002 Jun;20(2):87-93. Cd8(+)/V beta 5.1(+) large granular lymphocyte leukemia associated with autoimmune cytopenias, rheumatoid arthritis and vascular mammary skin lesions: successful response to 2-deoxycoformycin. Granjo E, Lima M, Correia T, Lisboa C, Magalhães C, Cunha N, Teixeira MA, Queirós ML, Candeias J, Matutes E. Department of Clinical Haematology, Hospital Geral de São João, Porto, Portugal. npp46740@mail.telepac.pt Abstract We report a case of CD8(+)/V beta 5.1(+) T-cell large granular lymphocyte leukemia (T-LGL leukemia) presenting with mild lymphocytosis, severe autoimmune neutropenia, thrombocytopenia, polyarthritis and recurrent infections with a chronic disease course. Immunophenotyping showed an expansion of CD3(+)/TCR alpha beta(+)/CD8(+bright)/CD11c(+)/CD57(-)/CD56(-) large granular lymphocytes with expression of the TCR-V beta 5.1 family. Southern blot analysis revealed a clonal rearrangement of the TCR beta-chain gene. Hematopoietic growth factors, high dose intravenous immunoglobulin and corticosteroids were of limited therapeutic benefit to correct the cytopenias. During the disease course, the patient developed a severe cutaneous leg ulcer and bilateral vascular mammary skin lesions. Treatment with 2-deoxycoformycin resulted in both clinical and hematological complete responses, including the resolution of vascular skin lesions. Combined immuno-staining with relevant T-cell associated and anti-TCR-V beta monoclonal antibodies proved to be a sensitive method to assess the therapeutic effect of 2-deoxycoformicin and to evaluate the residual disease. Copyright 2002 John Wiley & Sons, Ltd. PMID: 12111871 [PubMed - indexed for MEDLINE
Peer review: yes
URI: http://hdl.handle.net/10400.16/515
ISSN: 0278-0232
Versão do Editor: http://onlinelibrary.wiley.com/doi/10.1002/hon.695/abstract
Aparece nas colecções:SHC - Artigos publicados em revistas indexadas na Medline

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