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.

• Tipo: Outra Publicação em Revista Científica Internacional
• Data: 2002

Autores

Granjo, E (Autor) Outra; Lima, M (Autor) Outra; Correia, T (Autor) Outra; Carmen Silva (Autor) FMUP; Magalhaes, C (Autor) Outra; Cunha, N (Autor) Outra; Teixeira, MA (Autor) Outra; Queiros, ML (Autor) Outra; Candeias, J (Autor) Outra; Matutes, E (Autor) Outra

Revista

Título: Hematological Oncology

Vol. 20

Páginas: 87-93

ISSN: 0278-0232

Editora: Wiley-Blackwell

Indexação

ISI Web of Knowledge - 11 Citações

Scopus - 15 Citações

Outras Informações

ID Authenticus: P-000-P53

DOI: 10.1002/hon.695

Abstract (EN): We report a case of CD8(+)/Vbeta(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(+)/TCRalphabeta(+)/CD8(+bright)/CD11c(+)/CD57(-)/CD56(-) large granular lymphocytes with expression of the TCR-Vbeta5.1 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-Vbeta monoclonal antibodies proved to be a sensitive method to assess the therapeutic effect of 2-deoxycoformicin and to evaluate the residual disease. Copyright (C) 2002 John Wiley Sons, Ltd.

Idioma: Inglês

Tipo (Avaliação Docente): Científica

Nº de páginas: 7