T-cell receptor gammadelta T-cell leukemia with the morphology of T-cell prolymphocytic leukemia and a postthymic immunophenotype.

Abstract

T-cell prolymphocytic leukemia (T-PLL) is a postthymic T-cell neoplasm with a characteristic morphology and heterogeneous immunophenotype. Most cases of T-PLL express membrane T-cell receptors (TCRs) of the alphabeta phenotype. We experienced a 30-year-old man suffering from TCRgammadelta T-cell leukemia with morphology compatible to T-PLL with a postthymic phenotype. He was admitted with skin eruption and pancytopenia. Peripheral blood and bone marrow were occupied with medium-sized lymphocytes, which had moderately condensed chromatin with a single nucleolus and sparse, nongranular basophilic cytoplasm. The immunophenotype was CD1a-, CD2-, CD3+, CD4-, CD5+, CD7+, CD8-, and terminal deoxynucleotidyl transferase negative. Hepatosplenomegaly was absent. He was diagnosed as having T-PLL and was treated with combination chemotherapy. Six months later the leukemic cell became chemoresistant. Although the patient showed transient improvement in response to pentostatin, he died 13 months after the diagnosis. To our knowledge, this is the first case of T-PLL with a TCRgammadelta phenotype.

• Authors: T Sugimoto, S Imoto, Y Matsuo, K Kojima, M Yasukawa, T Murayama, J Kohfuku, I Mizuno, K Yakushijin, A Sada, R Nishimura, T Koizumi
• Journal: Annals of hematology (Ann Hematol) Vol. 80 Issue 12 Pg. 749-51 (Dec 2001) ISSN: 0939-5555 [Print] Germany
• PMID: 11797117 (Publication Type: Case Reports, Journal Article)

Chemical References

• Antigens, CD
• HLA-DR Antigens
• Receptors, Antigen, T-Cell, gamma-delta

Topics

• Adult
• Anemia
• Antigens, CD (analysis)
• Bone Marrow (pathology)
• Cell Membrane (immunology)
• Cytogenetic Analysis
• Erythema
• Flow Cytometry
• Gene Rearrangement, delta-Chain T-Cell Antigen Receptor
• Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor
• HLA-DR Antigens (analysis)
• Humans
• Immunophenotyping
• Leukemia, Prolymphocytic (diagnosis, immunology, pathology)
• Leukemia, T-Cell (diagnosis, immunology, pathology)
• Leukocyte Count
• Lymphatic Diseases
• Lymphocyte Count
• Male
• Microscopy, Electron
• Receptors, Antigen, T-Cell, gamma-delta (analysis, genetics)
• Thymus Gland (pathology)