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Lymphoproliferative disease of granular lymphocytes with T‐cell receptor gamma delta‐positive phenotype: restricted usage of T‐cell receptor gamma and delta subunit genes
Author(s) -
Makishima Hideki,
Ishida Fumihiro,
Saito Hiroshi,
Ichikawa Naoaki,
Ozaki Yayoi,
Ito Susumu,
Ota Masao,
Katsuyama Yoshihiko,
Kiyosawa Kendo
Publication year - 2003
Publication title -
european journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0902-4441
DOI - 10.1034/j.1600-0609.2003.00039.x
Subject(s) - t cell receptor , cd8 , immunophenotyping , cd3 , immunology , biology , cd16 , receptor , t cell , t lymphocyte , phenotype , gene , antigen , immune system , genetics
Lymphoproliferative disease of granular lymphocytes (LDGL) is characterized by more than 0.5 × 10 9 /L of proliferating granular lymphocytes in the peripheral blood. Because of its rarity, the characteristics of LDGL with T‐cell receptor (TCR) γ δ phenotype ( γ δ T‐LDGL) have not yet been identified. This report describes the clinical, hematological, and immunological findings of four patients with this disease. In two cases, the clinical course was indolent and the other two patients required various therapies. The cells had a common immunophenotype: CD3+, CD4–, CD16+, CD56–, CD57–, CD122–, TCR‐ γ δ +, and three were CD8‐positive. The immunopurified TCR‐ γ δ cells from the patients expressed only V γ 9 and V δ 1. Spectratyping and sequencing showed mono‐ or oligoclonality for TCR γ and TCR δ subunit genes. Soluble Fas ligand in sera was significantly elevated in all patients. These findings suggest that γ δ T‐LDGL qualifies as a distinct disease entity.