| Autor: |
Akira Ohara, Miyuki Kobayashi, Dario Campana, Koichiro Ikuta, Katsuyoshi Koh, Masahiro Tsuchida, Takeshi Inukai, Atsushi Manabe, Nobutaka Kiyokawa, Elaine Coustan-Smith, Kanji Sugita, Akira Kikuchi, Hiroyuki Takahashi, Yasuhide Hayashi, Masaaki Kumagai |
| Rok vydání: |
2011 |
| Předmět: |
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| Zdroj: |
British Journal of Haematology. 156:358-365 |
| ISSN: |
0007-1048 |
| Popis: |
Summary Early T-cell precursor acute lymphoblastic leukaemia (ETP-ALL) is a recently identified subtype of T-ALL with distinctive gene expression and cell marker profiles, poor response to chemotherapy and a very high risk of relapse. We determined the reliability of restricted panel of cell markers to identify EPT-ALL using a previously classified cohort. Then, we applied the cell marker profile that best discriminated ETP-ALL to a cohort of 91 patients with T-ALL enrolled in the Tokyo Children’s Cancer Study Group L99-15 study, which included allogeneic stem cell transplantation (allo-SCT) for patients with poor prednisone response. Five of the 91 patients (5·5%) met the ETP-ALL criteria. There were no significant differences in presenting clinical features between these and the remaining 86 patients. Response to early remission induction therapy was inferior in ETP-ALL as compared with T-ALL. The ETP-ALL subgroup showed a significantly poorer event-free survival (4-year rate; 40%) than the T-ALL subgroup (70%, P = 0·014). Of note, three of four relapsed ETP-ALL patients survived after allo-SCT, indicating that allo-SCT can be effective for this drug-resistant subtype of T-ALL. |
| Databáze: |
OpenAIRE |
| Externí odkaz: |
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