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

Journal of Pediatric Hematology/oncology
Asher M MoserJoseph Kapelushnik

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.

References

Sep 1, 1986·British Journal of Haematology·E MatutesD Catovsky
Aug 4, 1973·Lancet·D CatovskyG Stathopoulos
Jul 1, 1997·Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology·R PawsonD Catovsky
Feb 19, 2005·International Journal of Hematology·Shigeo ToyotaKazuo Dan
Apr 29, 2006·Medical Oncology·Claire E Dearden

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Citations

Dec 8, 2015·Hematology·Claire Dearden
Jan 14, 2017·International Journal of Dermatology·Geeti KhullarUma Nahar Saikia
Mar 28, 2017·Hematology/oncology Clinics of North America·Amit Sud, Claire Dearden

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Methods Mentioned

BETA
biopsy

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