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Open Access Case report

Pediatric T-cell prolymphocytic leukemia with an isolated 12(p13) deletion and aberrant CD117 expression

Michael Bellone1, Annika M Svensson1, Ann-Leslie Zaslav2, Silvia Spitzer3, Marc Golightly4, Mahmut Celiker5, Youjun Hu1, Yupo Ma16* and Tahmeena Ahmed16*

Author Affiliations

1 Department of Pathology, Stony Brook University Medical Center, Stony Brook, NY 11794, USA

2 Cytogenetics, Department of Pathology, Stony Brook University Medical Center, Stony Brook, NY 11794, USA

3 Molecular Genetics, Department of Pathology, Stony Brook University Medical Center, Stony Brook, NY 11794, USA

4 Flow Cytometry, Department of Pathology, Stony Brook University Medical Center, Stony Brook, NY 11794, USA

5 Department of Pediatrics, Stony Brook University Medical Center, Stony Brook, NY 11794, USA

6 Stony Brook University Medical Center, UH Level 2, Rm, 766, Stony Brook, NY 11794-7300, USA

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Experimental Hematology & Oncology 2012, 1:7  doi:10.1186/2162-3619-1-7

Published: 18 April 2012

Abstract

T-cell Prolymphocytic leukemia (T-PLL) is a rare post-thymic T-cell malignancy that follows an aggressive clinical course. The classical presentation includes an elevated white blood cell (WBC) count with anemia and thrombocytopenia, hepatosplenomegaly, and lymphadenopathy. T-PLL is a disease of the elderly and to our knowledge it has never been described in the pediatric age group. We report a case of T-PLL in a 9 year old male who was initially diagnosed with T-cell acute lymphoblastic lymphoma (ALL), the diagnosis was later refined to T-PLL following additional analysis of bone marrow morphology and immunophenotype. Two unusual findings in our patient included CD117 expression and an isolated chromosomal 12(p13) deletion. The patient failed to respond to standard ALL induction chemotherapy, but achieved complete remission following treatment with a fludarabine and alemtuzumab-based regimen.

Keywords:
T-cell Prolymphocytic Leukemia; Pediatric T-cell Lymphomas; Alemtuzumab; TCR rearrangement; CD117; 12p13