Lynch Ryan C, Advani Ranjana H
Division of Oncology, Department of Medicine, Stanford University School of Medicine, Stanford, California, USA.
Case Rep Oncol. 2017 Sep 12;10(3):813-818. doi: 10.1159/000480292. eCollection 2017 Sep-Dec.
The B-cell receptor signaling pathway is important in the lymphomagenesis of many lymphomas, including marginal zone lymphoma (MZL). Herein we describe a case of extranodal MZL refractory to multiple lines of therapy. The presence of an IgM paraprotein prompted further evaluation, and the patient was found to have an MYD88 mutation. Treatment with ibrutinib led to a dramatic response with prompt resolution of symptoms and significant improvement in measurable sites of disease. The excellent response to ibrutinib in our patient with MYD88-mutated refractory MZL supports a biological rationale for its use.
B细胞受体信号通路在包括边缘区淋巴瘤（MZL）在内的许多淋巴瘤的淋巴瘤发生过程中都很重要。在此，我们描述了一例对多线治疗均耐药的结外MZL病例。IgM副蛋白的存在促使进一步评估，结果发现该患者存在MYD88突变。使用伊布替尼治疗产生了显著反应，症状迅速缓解，疾病可测量部位有明显改善。我们这位MYD88突变的难治性MZL患者对伊布替尼的出色反应支持了其使用的生物学依据。
