[Report of six cases with mast cell leukemia and a literature review].

Zhonghua Nei Ke Za Zhi

Jiangsu Institute of Hematology, National Clinical Research Center for Hematologic Diseases, the First Affiliated Hospital of Soochow University, Suzhou 215006, China.

Published: October 2024

AI Article Synopsis

  • A retrospective analysis of six patients with acute mast cell leukemia (MCL) at Soochow University revealed a median age of 54, with various clinical presentations such as hypoalbuminemia, fatigue, and fever, highlighting the complexity of this rare disease.
  • Most patients displayed significant abnormalities in specific proteins (CD117, CD30, CD25, CD2) and gene mutations, indicating aggressive disease characteristics and a need for better diagnostic criteria.
  • Treatment outcomes were poor, with some patients receiving various therapies like venetoclax and cladribine, but only one achieved partial remission; this emphasizes the urgent need for improved understanding and treatment approaches for MCL.

Article 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 (=4), fatigue (=3), fever (=2), abdominal discomfort (=2), osteolytic lesions (=2), dizziness (=1), skin flushing (=1), and weight loss (=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.

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http://dx.doi.org/10.3760/cma.j.cn112138-20240401-00209DOI Listing

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