Report of six cases with mast cell leukemia and a literature review
10.3760/cma.j.cn112138-20240401-00209
- VernacularTitle:肥大细胞白血病6例并文献复习
- Author:
Feng ZHU
1
;
Yuan YU
;
Chunyan CHEN
;
Wenbing DUAN
;
Qian JIANG
;
Rui YAN
;
Yu SUN
;
Yanqiu HAN
;
Jing ZHANG
;
Huan WANG
;
Qiurong ZHANG
;
Suning CHEN
;
Wanhui YAN
;
Mengjie CAI
;
Zhibo ZHANG
;
Jia YIN
;
Qian WANG
Author Information
1. 苏州大学附属第一医院 江苏省血液研究所 国家血液系统疾病临床医学研究中心，苏州 215006
- Keywords:
Leukemia, mast-cell;
Prognosis;
Systemic mastocytosis;
Targeted therapy
- From:
Chinese Journal of Internal Medicine
2024;63(10):996-1000
- CountryChina
- Language:Chinese
-
Abstract:
From October 2021 to February 2023, we retrospectively analyzed the clinical and laboratory data of six patients (three male and three female, median age: 54 years, age range: 29-73 years) with mast cell leukemia (MCL) diagnosed in the First Affiliated Hospital of Soochow University (The Mastocytosis Collaborative Network of China). All patients had acute MCL, with at least one C-finding present. The main clinical presentations were hypoalbuminemia ( n=4), fatigue ( n=3), fever ( n=2), abdominal discomfort ( n=2), osteolytic lesions ( n=2), dizziness ( n=1), skin flushing ( n=1), and weight loss ( n=1). Splenomegaly and lymphadenopathy were noted in six and three patients, respectively. Six patients were strongly positive for CD117, five were positive for CD30 and CD25, and four were positive for CD2. Four patients had a normal karyotype and two patients had an abnormal karyotype. Gene mutations were detected in 4/6 cases. The median serum tryptase level was 24.9 (range: 20.1-171.9) μg/L. Two patients were treated with venetoclax and azacitidine for induction (one patient achieved partial remission by combination with afatinib, while there was no remission after combination with dasatinib in the other patient). Two patients did not achieve complete remission despite treatment with cladribine and imatinib, respectively. One patient treated with interferon combined with glucocorticoids was lost to follow-up, and one patient abandoned treatment. The follow-up time ranged from 1.1 to 21.7 months. Three patients died and two survived. Overall, MCL is a rare subtype of systemic mastocytosis with heterogeneous clinical course, and these patients have poor outcome. A better understanding of the clinical characteristics, treatment, and prognosis of MCL is urgently needed.
