A gamma/delta T-cell Receptor Prolymphocytic Leukemia and CD4(-)/CD8(-) Double-negative Immunophenotype in a Pediatric Patient

作者：Moser Asher M^*; Quider Abed A; Groen Jeremiah A; Shubinsky George; Kapelushnik Joseph

来源：Journal of Pediatric Hematology/Oncology, 2015, 37(4): E218-E219.

DOI：10.1097/mph.0000000000000317

• gamma/delta T-cell receptor
• T-cell prolymphocytic leukemia
• double-negative CD4(-)/CD8(-)
• pediatric

T-cell prolymphocytic leukemia is a very rare neoplasm, peaking in the seventh decade. An extensive search failed to find any report of this malignancy in the pediatric population. The malignant cell is morphologically characterized by a high nucleocytopasmic ratio, condensed chromatin, a single nucleolus, and nongranular basophilic cytoplasm. Cells are usually positive for the alpha/beta and only rarely to the gamma/delta T-cell receptors. Most patients follow an aggressive clinical course, only some respond to anti-CD52. We present a 6-year-old boy with T-cell prolymphocytic leukemia. The malignant cells expressed a postthymic immunophenotype (CD4(-)/CD8(-)) and positivity for the gamma/delta T-cell receptors. The child died after 8 months despite aggressive chemotherapy, anti-CD52, and an allogeneic bone marrow transplant.

• 出版日期2015-5