Pan Zhijuan, Zhang Ying, Guo Yanru, Sun Jiajia, Guo Xinlei, Guo Zhiping
Department of Hematology, Peking University First Hospital Taiyuan Branch (Taiyuan Central Hospital of Shanxi Medical University), No. 256, Fendong Street, Xiaodian District, Taiyuan, Shanxi 030009, PR China.
Leuk Res Rep. 2025 Apr 12;23:100510. doi: 10.1016/j.lrr.2025.100510. eCollection 2025.
Mast cell leukemia (MCL) is a rare and aggressive form of systemic mastocytosis with a poor prognosis. Understanding the different therapeutic responses to corticosteroids in MCL is crucial for improving patient outcomes.
We present a case of a 74-year-old Chinese female with primary acute MCL who exhibited different responses to dexamethasone and methylprednisolone. She was admitted with persistent fever, dyspnea, severe fatigue, and bone pain, alongside splenomegaly and cytopenia. Diagnosis was confirmed through marrow aspirate analysis, chemical staining, flow cytometry, and biopsy, revealing atypical mast cells positive for CD117, CD9, CD81, CD33, CD13, CD4, and partially for CD56, but negative for CD2 and CD25. Next-generation sequencing identified heterozygous mutations in , and 3, with no mutations. Initial treatment included corticosteroids and dasatinib. The patient showed a partial response to dexamethasone but significant improvement with methylprednisolone. Upon reintroduction of dexamethasone, symptoms recurred, which improved again after resuming methylprednisolone. The patient survived for three months post-diagnosis.
This case highlights the potential efficacy of methylprednisolone over dexamethasone in MCL treatment. This case underscores the importance of personalized treatment approaches in MCL, considering the distinct genetic profile and differential therapeutic responses to corticosteroids. Further research is needed to elucidate the mechanisms underlying these responses and to optimize treatment strategies for MCL.
肥大细胞白血病（MCL）是系统性肥大细胞增多症的一种罕见且侵袭性的形式，预后较差。了解MCL对皮质类固醇的不同治疗反应对于改善患者预后至关重要。
我们报告一例74岁中国女性原发性急性MCL患者，其对地塞米松和甲泼尼龙表现出不同反应。她因持续发热、呼吸困难、严重疲劳和骨痛入院，伴有脾肿大和血细胞减少。通过骨髓穿刺分析、化学染色、流式细胞术和活检确诊，显示非典型肥大细胞CD117、CD9、CD81、CD33、CD13、CD4阳性，部分CD56阳性，但CD2和CD25阴性。二代测序在 、 和3中鉴定出杂合突变，无 突变。初始治疗包括皮质类固醇和达沙替尼。患者对地塞米松有部分反应，但对甲泼尼龙有显著改善。再次使用地塞米松后症状复发，恢复甲泼尼龙治疗后症状再次改善。患者确诊后存活了三个月。
该病例突出了甲泼尼龙在MCL治疗中优于地塞米松的潜在疗效。该病例强调了在MCL中采用个性化治疗方法的重要性，考虑到独特的基因特征和对皮质类固醇的不同治疗反应。需要进一步研究以阐明这些反应的潜在机制，并优化MCL的治疗策略。
