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Prognostic significance of cytogenetic abnormalities in T‐cell prolymphocytic leukemia
Author(s) -
Hu Zhihong,
Medeiros L. Jeffrey,
Fang Lianghua,
Sun Yi,
Tang Zhenya,
Tang Guilin,
Sun Tsieh,
Quesada Andres E.,
Hu Shimin,
Wang Sa A.,
Pei Lin,
Lu Xinyan
Publication year - 2017
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.24679
Subject(s) - prolymphocytic leukemia , karyotype , gastroenterology , fluorescence in situ hybridization , medicine , cytogenetics , leukemia , gene rearrangement , transplantation , abnormality , biology , pathology , chromosome , chronic lymphocytic leukemia , genetics , psychiatry , gene
T‐cell prolymphocytic leukemia (T‐PLL) is an aggressive mature T‐cell neoplasm. The most common cytogenetic abnormality associated with T‐PLL is inv(14)(q11.2q32) involving TCL1 , but other abnormalities also have been reported. In this study, we correlated cytogenetic abnormalities with clinical outcome in 97 T‐PLL patients, including 66 men and 31 women with a median age of 63 years (range, 34‐81). Twenty‐seven patients had a normal karyotype (NK), one had two chromosomal aberrations, and 69 had a complex karyotype (CK). Patients with a CK had poorer overall survival (OS) than patients with a NK ( P  = .0016). In the CK group, the most common aberrations involved 14q (n = 45) and 8q (n = 38). Additional deletions of chromosomes 17p, 11q, 6q, 12p, 13q were observed frequently. No individual cytogenetic abnormality impacted OS. Patients with ≥5 aberrations had an OS of 11 months versus 22 months in patients with <5 aberrations ( P  = 0.0132). Fluorescence in situ hybridization for TCL1 successfully performed in 27 cases showed rearrangement in 8/10 (80%) NK versus 16/17 (94%) CK cases. OS of patients with TCL1 rearrangement and/or 14q aberrations was not significantly different from patients without TCL1 rearrangement and 14q aberrations ( P  = .3467). Patients with refractory disease showed worse OS in both the NK and CK groups ( P  = .0014 and P  < .0001, respectively), compared with patients who achieved remission but then relapsed. Stem cell transplantation did not appear to improve OS regardless of karyotype complexity. In conclusion, patients with T‐PLL often have a CK which is a poor prognostic factor, particularly in patients with ≥5 cytogenetic aberrations.

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