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.

• Type: Another Publication in an International Scientific Journal
• Date: 2002

Authors

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

Journal

Title: Hematological Oncology

Vol. 20

Pages: 87-93

ISSN: 0278-0232

Publisher: Wiley-Blackwell

Indexing

ISI Web of Knowledge - 11 Citations

Scopus - 15 Citations

Other information

Authenticus ID: 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.

Language: English

Type (Professor's evaluation): Scientific

No. of pages: 7