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.

摘要: 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. We report a case of CD8(+)/Vbeta(5.1)(+) T-cell (T-LGL leukemia) presenting mild lymphocytosis, severe neutropenia, thrombocytopenia, polyarthritis recurrent infections chronic disease course. Immunophenotyping showed an expansion CD3(+)/TCRalphabeta(+)/CD8(+bright)/CD11c(+)/CD57(-)/CD56(-) lymphocytes expression the TCR-Vbeta5.1 family. Southern blot analysis revealed clonal rearrangement TCR beta-chain gene. Hematopoietic growth factors, high dose intravenous immunoglobulin corticosteroids were limited therapeutic benefit correct cytopenias. During course, patient developed cutaneous leg ulcer bilateral lesions. Treatment 2-deoxycoformycin resulted in both clinical hematological complete responses, including resolution Combined immuno-staining relevant anti-TCR-Vbeta monoclonal antibodies proved be sensitive method assess effect 2-deoxycoformicin evaluate residual disease.