Abstract：
BACKGROUND: The coexistence of the extranidal marginal zone lymphoma (MZL) of mucosa-associated lymphoid tissue (MALT) and multiple myeloma (MM) is an exceedingly rare situation. The rare situation precludes any evidence-based guidelines for MZL or MM.
METHODS: We presented a unique case of the coexistence of primary mediastinal MALT lymphoma and MM like polyneuropathy, organomegaly, endocrinopathy, M-protein, skin syndrome.
RESULTS: The patient was first diagnosed with polyneuropathy, organomegaly, endocrinopathy, M-protein, skin syndrome in the department of neurology, then MM in the department of hematology, and the mediastinal MALT simultaneously coexisting with MM was found by biopsy in the department of thoracic surgery. The patient received combination therapy with rituximab and bortezomib followed by lenalidomide maintenance. To understand MZL lymphoma with plasmacytic differentiation better, we analyzed cases of MZL lymphomas with plasma cell neoplasms. Most of these cases were MZL lymphomas with light chain-restricted plasmacytic differentiation. The lymphomas relapsed with plasma cell neoplasms or transformed into plasma cell neoplasms after anti-lymphoma therapy.
CONCLUSIONS: The case demonstrated clinical complexity and the importance of the detailed assessment. The case and literature review demonstrated the value of detecting light chain-restricted plasmacytic differentiation for the treatment of MZL lymphoma with rituximab plus lenalidomide or bortezomib.
METHODS: We presented a unique case of the coexistence of primary mediastinal MALT lymphoma and MM like polyneuropathy, organomegaly, endocrinopathy, M-protein, skin syndrome.
RESULTS: The patient was first diagnosed with polyneuropathy, organomegaly, endocrinopathy, M-protein, skin syndrome in the department of neurology, then MM in the department of hematology, and the mediastinal MALT simultaneously coexisting with MM was found by biopsy in the department of thoracic surgery. The patient received combination therapy with rituximab and bortezomib followed by lenalidomide maintenance. To understand MZL lymphoma with plasmacytic differentiation better, we analyzed cases of MZL lymphomas with plasma cell neoplasms. Most of these cases were MZL lymphomas with light chain-restricted plasmacytic differentiation. The lymphomas relapsed with plasma cell neoplasms or transformed into plasma cell neoplasms after anti-lymphoma therapy.
CONCLUSIONS: The case demonstrated clinical complexity and the importance of the detailed assessment. The case and literature review demonstrated the value of detecting light chain-restricted plasmacytic differentiation for the treatment of MZL lymphoma with rituximab plus lenalidomide or bortezomib.
摘要：
背景：粘膜相关淋巴样组织（MALT）的外边缘区淋巴瘤（MZL）和多发性骨髓瘤（MM）的共存是非常罕见的情况。这种罕见的情况排除了MZL或MM的任何循证指南。
方法：我们介绍了原发性纵隔MALT淋巴瘤和MM样多发性神经病并存的独特病例，器官肿大,内分泌病,M-蛋白,皮肤综合征。
结果：患者最初被诊断为多发性神经病，器官肿大,内分泌病,M-蛋白,神经内科的皮肤综合征，然后是血液科的MM,胸外科活检发现纵隔MALT与MM同时存在。患者接受利妥昔单抗和硼替佐米联合治疗，然后进行来那度胺维持治疗。为了更好地了解MZL淋巴瘤的浆细胞分化，我们分析了MZL淋巴瘤伴浆细胞肿瘤的病例。这些病例大多数是具有轻链限制性浆细胞分化的MZL淋巴瘤。在抗淋巴瘤治疗后，淋巴瘤随浆细胞肿瘤复发或转化为浆细胞肿瘤。
结论：该病例证明了临床复杂性和详细评估的重要性。该病例和文献综述证明了检测轻链限制性浆细胞分化对利妥昔单抗联合来那度胺或硼替佐米治疗MZL淋巴瘤的价值。
方法：我们介绍了原发性纵隔MALT淋巴瘤和MM样多发性神经病并存的独特病例，器官肿大,内分泌病,M-蛋白,皮肤综合征。
结果：患者最初被诊断为多发性神经病，器官肿大,内分泌病,M-蛋白,神经内科的皮肤综合征，然后是血液科的MM,胸外科活检发现纵隔MALT与MM同时存在。患者接受利妥昔单抗和硼替佐米联合治疗，然后进行来那度胺维持治疗。为了更好地了解MZL淋巴瘤的浆细胞分化，我们分析了MZL淋巴瘤伴浆细胞肿瘤的病例。这些病例大多数是具有轻链限制性浆细胞分化的MZL淋巴瘤。在抗淋巴瘤治疗后，淋巴瘤随浆细胞肿瘤复发或转化为浆细胞肿瘤。
结论：该病例证明了临床复杂性和详细评估的重要性。该病例和文献综述证明了检测轻链限制性浆细胞分化对利妥昔单抗联合来那度胺或硼替佐米治疗MZL淋巴瘤的价值。
