奥马珠单抗治疗惰性系统性肥大细胞增多症的疗效
Efficacy of Omalizumab in Indolent Systemic Mastocytosis.
作者信息
Slapnicar Calum, Trinkaus Martina, Hicks Lisa, Vadas Peter
机构信息
Division of Allergy and Clinical Immunology, St. Michael's Hospital, University of Toronto, Toronto, Ontario, Canada.
Division of Hematology, St. Michael's Hospital, University of Toronto, Toronto, Ontario, Canada.
出版信息
Case Rep Hematol. 2019 Sep 16;2019:3787586. doi: 10.1155/2019/3787586. eCollection 2019.
BACKGROUND
Systemic mastocytosis (SM) comprises a heterogeneous group of disorders characterized by the proliferation of clonal mast cells in skin and various internal organs. Omalizumab is an established, labelled therapy for allergic asthma and chronic urticaria, but its experience in the efficacy of SM is limited.
METHODS
A retrospective analysis of 6 patients diagnosed with indolent SM treated with omalizumab at St. Michael's Hospital between 2009 and 2018 is described. Reported frequency of anaphylaxis, baseline and follow-up tryptase levels, and SM-related symptoms were captured to measure the control of the disease.
RESULTS
Of the 5 patients who had experienced unprovoked anaphylaxis prior to treatment with omalizumab, 3 had no further episodes of anaphylaxis following initiation of omalizumab, while the remaining 2 patients had milder multisystem reactions. Significant improvement in cutaneous symptoms was also observed.
CONCLUSION
These data suggest that omalizumab provides benefit to patients with SM who remain highly symptomatic in spite of treatment with conventional therapies.
背景
系统性肥大细胞增多症（SM）是一组异质性疾病，其特征为皮肤和各种内脏器官中克隆性肥大细胞增殖。奥马珠单抗是一种已获批的用于治疗过敏性哮喘和慢性荨麻疹的药物，但其在SM疗效方面的经验有限。
方法
描述了对2009年至2018年间在圣迈克尔医院接受奥马珠单抗治疗的6例惰性SM患者的回顾性分析。记录过敏反应的报告频率、基线和随访时的类胰蛋白酶水平以及与SM相关的症状，以评估疾病的控制情况。
结果
在奥马珠单抗治疗前经历过自发性过敏反应的5例患者中，3例在开始使用奥马珠单抗后未再发生过敏反应，其余2例患者出现较轻的多系统反应。皮肤症状也有显著改善。
结论
这些数据表明，对于尽管接受了传统治疗仍有高度症状的SM患者，奥马珠单抗可带来益处。
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