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(+)/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.
Department of Clinical Haematology, Hospital Geral de São João, Porto, Portugal. npp46740@mail.telepac.pt
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E
Granjo
M
M
Lima
T
T
Correia
C
C
Lisboa
C
C
Magalhães
N
N
Cunha
M A
MA
Teixeira
M L
ML
Queirós
J
J
Candeias
E
E
Matutes
D000368:Aged; D000903:Antibiotics, Antineoplastic / Q000627:therapeutic use*; D001172:Arthritis, Rheumatoid / Q000150:complications*; D001940:Breast / Q000098:blood supply; D017252:CD3 Complex / Q000032:analysis; D016827:CD8 Antigens; D005260:Female; D015329:Gene Rearrangement, T-Lymphocyte / Q000276:immunology; D006801:Humans; D016130:Immunophenotyping; D007871:Leg Ulcer / Q000150:complications; D007945:Leukemia, Lymphoid / Q000150:complications / Q000188:drug therapy* / Q000276:immunology*; D009503:Neutropenia / Q000150:complications; D015649:Pentostatin / Q000627:therapeutic use*; D016693:Receptors, Antigen, T-Cell, alpha-beta / Q000276:immunology; D013921:Thrombocytopenia / Q000150:complications
D000903:Antibiotics, Antineoplastic; D017252:CD3 Complex; D016827:CD8 Antigens; D016693:Receptors, Antigen, T-Cell, alpha-beta; D015649:Pentostatin