Clinical Characterization of Mogamulizumab-Associated Rash During Treatment of Mycosis Fungoides or Sezary Syndrome
Clinical Characterization of Mogamulizumab-Associated Rash During Treatment of Mycosis Fungoides or Sezary Syndrome
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DOI：
10.1001/jamadermatol.2021.0877
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发表时间：
2021-04-21
期刊：
影响因子：
10.9
通讯作者：
Kwong, Bernice Y.
中科院分区：
文献类型：
--
作者：
Hirotsu, Kelsey E.;Neal, Tatiana M.;Kwong, Bernice Y.
重要性 Mogamulizumab 是一种针对 CCR4 的单克隆抗体，已被批准用于治疗蕈样肉芽肿 (MF) 和塞扎里综合征 (SS)。 Mogamulizumab 相关皮疹 (MAR) 很难与皮肤 MF 或 SS 区分开来，这可能会导致不必要的停药，因为担心严重药物反应或错误地推测皮肤疾病复发或进展。 目的 检查 MF 或 SS 患者中最常见的 MAR 临床表现以及诊断和管理挑战。 设计、设置和参与者 本回顾性病例系列评估了多学科皮肤淋巴瘤患者诊断为 MF 或 SS 并在 2013 年 1 月 1 日至 2020 年 1 月 1 日期间在主要学术转诊中心的诊所和支持性肿瘤病学诊所接受 mogamulizumab 治疗。治疗后出现新发或恶化的皮疹，皮肤活检结果与通过临床病理相关性和分子检测确定的药疹一致，以排除活动性恶性疾病。暴露至少 1 剂 mogamulizumab。主要结果和措施Mogamulizumab 相关皮疹的特征是临床特征，包括发病时间、临床表现、组织病理学特征和治疗方法。 结果 该研究包括 19 名发生 MAR 的 MF 或 SS 患者（中位年龄，65 岁；年龄范围，38-82 岁；10 名 [52.6%] 男性）。至 MAR 发病的中位时间为 119 天（范围为 56 天至 3.8 年）。 MAR 患者表现出 4 种主要临床表现：(1) 毛囊性 MF 样头皮斑块伴脱发，(2) 丘疹和/或斑块，(3) 光致加重皮炎，(4) 麻疹样皮炎或红皮病皮炎。最常见的解剖区域是头部和颈部，包括头皮。组织病理学结果各不相同，并且与主要临床形态学结果不相符。免疫组织化学和 T 细胞克隆性辅助检测有助于区分 MAR 和疾病。大多数 MAR 患者（19 名患者中的 14 名）停止了 mogamulizumab 治疗；然而，没有发生危及生命的严重皮肤药物不良反应，并且停止药物治疗的决定通常是多因素的。四名患者再次接受 mogamulizumab 治疗，没有出现危及生命的药物相关事件。 MAR 的治疗方法包括局部皮质类固醇、全身性皮质类固醇和/或甲氨蝶呤。 结论和相关性 本病例系列发现，莫加穆利珠单抗相关皮疹在 MF 或 SS 患者中具有异质性临床表现，其临床表现各异且起病延迟。 Mogamulizumab 相关皮疹好发于头颈部，在临床上很难与疾病复发或进展区分开来。识别最常见的临床表现有助于防止不必要地停止莫加穆利珠单抗治疗。 MAR 的存在并不需要永久停止或避免 mogamulizumab 的再治疗。
IMPORTANCE Mogamulizumab is a monoclonal antibody against CCR4 approved for treatment for mycosis fungoides (MF) and Sezary syndrome (SS). Mogamulizumab-associated rash (MAR) is difficult to differentiate from cutaneous MF or SS, which can lead to unnecessary discontinuation of drug use because of concern for severe drug reaction or incorrect presumption of disease relapse or progression in the skin.OBJECTIVE To examine the most common clinical presentations of MAR in patients with MF or SS and the diagnostic and management challenges.DESIGN, SETTING, AND PARTICIPANTS This retrospective case series assessed patients from a multidisciplinary cutaneous lymphoma clinic and supportive oncodermatology clinic at a major academic referral center who had a diagnosis of MF or SS and received mogamulizumab from January 1, 2013, to January 1, 2020. Treatment was followed by new or worsening rash with skin biopsy results compatible with drug eruption determined by clinicopathologic correlation and molecular testing to exclude active malignant disease.EXPOSURES At least 1 dose of mogamulizumab.MAIN OUTCOMES AND MEASURES Mogamulizumab-associated rash was characterized by clinical features, including time to onset, clinical presentation, histopathologic features, and management approach.RESULTS The study included 19 patients with MF or SS who developed MAR (median age, 65 years; age range, 38-82 years; 10 [52.6%] male). Median time to MAR onset was 119 days (range, 56 days to 3.8 years). Patients with MAR exhibited 4 predominant clinical presentations: (1) folliculotropic MF-like scalp plaques with alopecia, (2) papules and/or plaques, (3) photoaccentuated dermatitis, and (4) morbilliform or erythrodermic dermatitis. The most common anatomical region involved was the head and neck, including the scalp. Histopathologic findings were variable and did not correspond to primary clinical morphologic findings. Immunohistochemistry and T-cell clonality ancillary testing were helpful to distinguish MAR from disease. Most patients with MAR (14 of 19) discontinued mogamulizumab treatment; however, no life-threatening severe cutaneous adverse drug reactions occurred, and the decision for drug therapy cessation was usually multifactorial. Four patients were treated again with mogamulizumab with no life-threatening drug-related events. Approaches to management of MAR include topical corticosteroids, systemic corticosteroids, and/or methotrexate.CONCLUSIONS AND RELEVANCE This case series found that mogamulizumab-associated rash had a heterogeneous clinical presentation with variable and delayed onset in patients with MF or SS. Mogamulizumab-associated rash exhibited a predilection for the head and neck and was difficult to clinically distinguish from relapse or progression of disease. Recognition of the most common clinical presentations can help prevent unnecessary discontinuation of mogamulizumab treatment. The presence of MAR does not necessitate permanent discontinuation of or avoidance of retreatment with mogamulizumab.