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(a) principal: | |
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Data de Publicação: | 2002 |
Outros Autores: | , , , , , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
Texto Completo: | http://hdl.handle.net/10400.16/515 |
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 |
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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.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 MEDLINEWiley-BlackwellRepositório Científico do Centro Hospitalar Universitário de Santo AntónioGRANJO, E.LIMA, M.CORREIA, T.LISBOA, C.MAGALHAES, CCUNHA, N.TEIXEIRA, M.A.QUEIROS, M.L.2011-01-19T10:38:48Z2002-062002-06-01T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttp://hdl.handle.net/10400.16/515eng0278-0232info:eu-repo/semantics/openAccessreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAP2023-10-20T10:52:50Zoai:repositorio.chporto.pt:10400.16/515Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T20:36:34.532464Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse |
dc.title.none.fl_str_mv |
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. |
title |
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. |
spellingShingle |
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. |
title_short |
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. |
title_full |
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. |
title_fullStr |
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. |
title_full_unstemmed |
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. |
title_sort |
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. |
author |
GRANJO, E. |
author_facet |
GRANJO, E. LIMA, M. CORREIA, T. LISBOA, C. MAGALHAES, C CUNHA, N. TEIXEIRA, M.A. QUEIROS, M.L. |
author_role |
author |
author2 |
LIMA, M. CORREIA, T. LISBOA, C. MAGALHAES, C CUNHA, N. TEIXEIRA, M.A. QUEIROS, M.L. |
author2_role |
author author author author author author author |
dc.contributor.none.fl_str_mv |
Repositório Científico do Centro Hospitalar Universitário de Santo António |
dc.contributor.author.fl_str_mv |
GRANJO, E. LIMA, M. CORREIA, T. LISBOA, C. MAGALHAES, C CUNHA, N. TEIXEIRA, M.A. QUEIROS, M.L. |
description |
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 |
publishDate |
2002 |
dc.date.none.fl_str_mv |
2002-06 2002-06-01T00:00:00Z 2011-01-19T10:38:48Z |
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info:eu-repo/semantics/publishedVersion |
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info:eu-repo/semantics/article |
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http://hdl.handle.net/10400.16/515 |
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eng |
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0278-0232 |
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Wiley-Blackwell |
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Wiley-Blackwell |
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