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.
Subject(s)
Immunophenotyping , Leukemia, Prolymphocytic/immunology , Leukemia, Prolymphocytic/pathology , Leukemia, T-Cell/immunology , Leukemia, T-Cell/pathology , Receptors, Antigen, T-Cell, gamma-delta/analysis , 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 , Leukemia, Prolymphocytic/diagnosis , Leukemia, T-Cell/diagnosis , Leukocyte Count , Lymphatic Diseases , Lymphocyte Count , Male , Microscopy, Electron , Receptors, Antigen, T-Cell, gamma-delta/genetics , Thymus Gland/pathologyABSTRACT
A 51-year-old man was admitted for treatment of severe thrombocytopenia in May 1997. A diagnosis of MDS RA (refractory thrombocytopenia; RTC) was made by bone marrow examination, which revealed mild marrow hypoplasia and a reduced number of megakaryocytes accompanied by micromegakaryocytes and hypolobular megakaryocytes. Chromosome analysis demonstrated 46, XY, t(5;7) (q31;q22) in all 20 cells examined. The patient received only supportive therapy including platelet transfusion, until leukocytosis and monocytosis gradually developed in November 1998. In view of a marked increase in the number of monocytes (more than 3,000/microliter), a diagnosis of CMML was made in December 1998. As the leukocytosis progressed, various inflammatory symptoms such as facial erythema and endophthalmitis developed. Administration of interferon alpha (IFN alpha) unexpectedly worsened the leukocytosis and monocytosis, suggesting abnormal responses of these cells to IFN alpha. Detailed molecular analysis of these cells might reveal a novel mechanism of leukemogenesis associated with 5q31.
