Chronic clonal proliferative disease of gamma-delta (gammadelta) T-cells in a patient with rheumatoid arthritis and neutropenia: lack of the morphology and the immunophenotype of large granular lymphocytes.
Normal gammadelta T-lymphocytes have the morphology of large granular lymphocytes (LGL) and, as the LGL of alphabeta T-cells, they express pan-T antigens, NK-associated antigens and the cytotoxic molecules, perforin and granzime B. In this report we describe an unusual patient with rheumatoid arthritis and neutropenia who has a chronic gammadelta T-cell proliferation with a chronic, indolent clinical course and atypical lymphocytes, lacking the classical LGL morphology, not expressing NK-associated antigens, and not expressing perforin or granzyme B. In spite of the atypical morphological features of the clonal cells, which were suggestive of a more malignant process, the patient has been followed for 4 years without aggressive therapy. It is important to recognize this entity and to distinguish it from other gammadelta T proliferations such as the hepatosplenic gammadelta T-cell lymphoma.
Sánchez
I Pérez
IP
Department of Haematology, Hospital General Gregorio Marañón, Madrid, Spain.
Longo
J López
JL
Soto
A Escudero
AE
Herrera
J Gil
JG
Velaso
J Anguita
JA
Casanova
M
M
Cros
T Pintado
TP
D000328:Adult; D000368:Aged; D001172:Arthritis, Rheumatoid / Q000150:complications* / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D001853:Bone Marrow / Q000473:pathology; D049109:Cell Proliferation; D002908:Chronic Disease; D002999:Clone Cells / Q000276:immunology / Q000378:metabolism / Q000473:pathology; D006801:Humans; D016130:Immunophenotyping; D007945:Leukemia, Lymphoid / Q000150:complications* / Q000276:immunology / Q000473:pathology*; D008297:Male; D009503:Neutropenia / Q000150:complications* / Q000276:immunology / Q000473:pathology; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000378:metabolism*; D013601:T-Lymphocytes / Q000276:immunology / Q000378:metabolism* / Q000473:pathology*
D016692:Receptors, Antigen, T-Cell, gamma-delta