Small Cell Variant of T-Cell Prolymphocytic Leukemia with a 33 Immunophenotype

Abstract

T-cell prolymphocytic leukemia (T-PLL) is a rare postthymic T-cell disorder. The disease is characterized by lymphadenopathy, splenomegaly, skin lesions, a high white blood cell count, and an aggressive clinical course. The small cell variant of T-PLL occurs in approximately 20% of patients. Most T-PLL patients express membrane T-cell receptors (TCR) of the alphabeta phenotype. The diagnosis of small cell variant T-PLL in a 56-year-old woman was based on the findings of abnormal lymphocytosis, immunophenotype, lymphadenopathy, and aggressive clinical behavior. Immunophenotype analysis showed that lymphocytes were positive for CD2, CD3, CD5, CD7, CD8, and TCR gammadelta antigens and negative for CD1a, CD4, and TCR alphabeta antigens. Southern blot analysis revealed rearrangement of the TCR Jgamma and Jdelta-1 genes. A cytogenetic study of peripheral blood showed a normal karyotype. T-PLL with a TCR gammadelta phenotype is very rare. This case was typical T-PLL except for the morphologically small cell type and the lack of the typical chromosome aberration. If cases accumulate in the future, the specific features of the gamma8 type of T-PLL will become clearer.

TCL1A gene involvement in T-cell prolymphocytic leukemia in Japanese patients

“…The frequency of 14q32 abnormalities on conventional cytogenetic analysis ranged from 25 to 76.6% in cases with T-PLL [1][2][3]. A total of 30 T-PLL cases, including 11 in the present study, have been reported in Japan [21,[26][27][28][29][30][31][32][33][34]; however, the frequency of typical chromosomal abnormalities involving 14q32 and Xq28 is low (3.3%) according to conventional methods. These data confirm a previous report [21].…”

T-cell prolymphocytic leukemia: An aggressive T cell malignancy with frequent cutaneous tropism

“…However, because of the aggressive clinical course that defines most cases of T-PLL, it was reclassified by the World Health Organization (WHO). [1][2][3][4][5][6] Among the classic clinical features are lymphocytosis, lymphadenopathy, cutaneous disease, and splenomegaly. 3,5,6 One of the characteristic features of T-PLL is cutaneous involvement; the reported incidence is between 25% and 30%.…”

A Case Report of T Cell Prolymphocytic Leukemia and Kaposi Sarcoma and a Review of T Cell Prolymphocytic Leukemia

“…Clonal rearrangement of T cell receptors is present in all cases of T-PLL with TCRαβ phenotypes being the most common. TCRγδ phenotypes have been reported in rare instances [11,12]. …”