| Title: |
Pediatric T-cell prolymphocytic leukemia with an isolated 12(p13) deletion and aberrant CD117 expression |
| Authors: |
Bellone Michael, Svensson Annika M, Zaslav Ann-Leslie, Spitzer Silvia, Golightly Marc, Celiker Mahmut, Hu Youjun, Ma Yupo, Ahmed Tahmeena |
| Source: |
Experimental Hematology & Oncology, Vol 1, Iss 1, p 7 (2012) |
| Publisher Information: |
BMC, 2012. |
| Publication Year: |
2012 |
| Collection: |
LCC:Diseases of the blood and blood-forming organs
LCC:Neoplasms. Tumors. Oncology. Including cancer and carcinogens |
| Subject Terms: |
T-cell Prolymphocytic Leukemia, Pediatric T-cell Lymphomas, Alemtuzumab, TCR rearrangement, CD117, 12p13, Diseases of the blood and blood-forming organs, RC633-647.5, Neoplasms. Tumors. Oncology. Including cancer and carcinogens, RC254-282 |
| More Details: |
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. |
| Document Type: |
article |
| File Description: |
electronic resource |
| Language: |
English |
| ISSN: |
2162-3619 |
| Relation: |
http://www.ehoonline.org/content/1/1/7; https://doaj.org/toc/2162-3619 |
| DOI: |
10.1186/2162-3619-1-7 |
| Access URL: |
https://doaj.org/article/f98347194c7e4d20a2dc00ecc37b277d |
| Accession Number: |
edsdoj.f98347194c7e4d20a2dc00ecc37b277d |
| Database: |
Directory of Open Access Journals |
