A γ/δ T-cell receptor prolymphocytic leukemia and CD4-/CD8- double-negative immunophenotype in a pediatric patient

J Pediatr Hematol Oncol. 2015 May;37(4):e218-9. doi: 10.1097/MPH.0000000000000317.

Abstract

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 α/β and only rarely to the γ/δ 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 γ/δ T-cell receptors. The child died after 8 months despite aggressive chemotherapy, anti-CD52, and an allogeneic bone marrow transplant.

Publication types

  • Case Reports

MeSH terms

  • Antigens, CD / analysis*
  • CD8 Antigens / analysis*
  • Child
  • Humans
  • Immunophenotyping
  • Leukemia, Prolymphocytic, T-Cell / immunology*
  • Male
  • Receptors, Antigen, T-Cell, gamma-delta / analysis*

Substances

  • Antigens, CD
  • CD8 Antigens
  • Receptors, Antigen, T-Cell, gamma-delta