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

• Published in: Experimental hematology & oncology Vol. 1; no. 1; p. 7
• Main Authors: Bellone, Michael, Svensson, Annika M, Zaslav, Ann-Leslie, Spitzer, Silvia, Golightly, Marc, Celiker, Mahmut, Hu, Youjun, Ma, Yupo, Ahmed, Tahmeena
• Format: Journal Article
• Language: English
• Published: England BioMed Central Ltd 18.04.2012; BioMed Central; BMC
• Subjects: 12p13; Alemtuzumab; Analysis; Anemia; Blood cell count; Cancer; Case Report; Case studies; CD117; Chemotherapy; Chromosome abnormalities; Complications and side effects; Diagnosis; Genetic aspects; Health aspects; Leukemia; Medical research; Medicine, Experimental; Non-Hodgkin's lymphomas; Pediatric T-cell Lymphomas; Pediatrics; Risk factors; T cells; T-cell Prolymphocytic Leukemia; TCR rearrangement; United States
• ISSN: 2162-3619; 2162-3619
• DOI: 10.1186/2162-3619-1-7

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.