Clinical analysis of 20 cases of small B lymphocyte proliferative disease with t (14;19) (q32;q13)
10.3760/cma.j.issn.0253-2727.2022.08.010
- VernacularTitle:伴t(14;19)(q32;q13)小B淋巴细胞增殖性疾病20例临床分析
- Author:
Hui YANG
1
;
Rui GUO
;
Yu SHI
;
Chun QIAO
;
Yujie WU
;
Lei FAN
;
Wei XU
;
Kourong MIAO
;
Jianyong LI
;
Hairong QIU
Author Information
1. 南京医科大学第一附属医院，江苏省人民医院血液科，南京 210029
- Keywords:
Leukemia, lymphocytic, chronic;
t (14;
19) (q32;
q13);
IGH/BCL3;
Clinical features;
Prognosis
- From:
Chinese Journal of Hematology
2022;43(8):674-679
- CountryChina
- Language:Chinese
-
Abstract:
Objective:The clinical characteristics and prognosis of 20 patients with small B-lymphocyte proliferative disease with t (14;19) (q32; q13) were analyzed to improve the understanding of such rare cases.Methods:The clinical data of 20 patients with t (14; 19) (q32; q13) small B lymphocyte proliferative disease treated in the First Affiliated Hospital of Nanjing Medical University from April 2013 to December 2020 were retrospectively collected and analyzed. Among them, 10 cases were chronic lymphocytic leukemia (CLL) and 10 cases were other small B-cell malignancies.Results:Among the 20 cases, 10 were male and 10 were female, and the median age at diagnosis was 53.5 (35-88) years old. All patients had absolute lymphocytosis, 19 patients had lymphadenopathy, and 10 patients had splenomegaly. With a median follow-up of 36 (4-163) months, three patients died, and 11 patients had a time to treatment (TTT) ≤12 months. Ten patients (50%) were accompanied by +12, two patients (2/17, 12%) were accompanied by 13q-. Moreover, we found that t (14;19) was associated with unmutated immunoglobulin heavy-chain variable (IGHV) somatic mutation (17/19, 89%) and a biased use of IGHV4-39 (7/17, 41%) was observed. Next-generation sequencing detected one or more gene mutations in 14 (14/17, 82%) cases and a total of 25 gene mutations had been revealed, of which the most frequent were NOTCH1 (35%) , followed by SF3B1 (24%) and KMT2D (18%) . For 10 CLL patients, five (50%) were defined as Rai Ⅲ/Binet C. It is noteworthy that among the 20 cases, two cases actually involved Richter transformation.Conclusions:Small B-cell malignant tumors with abnormal t (14; 19) show unique clinical biological characteristics, often accompanied by a variety of adverse prognostic factors, and tend to have an aggressive clinical course.
