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Abstract：
BACKGROUND: The co-existence of Waldenström\'s macroglobulinemia (WM) with internodal marginal zone lymphoma (INMZL) is rare and often associated with poor prognosis.
METHODS: We present a Chinese female patient who developed secondary light chain amyloidosis due to WM and INMZL and provides opinions on its systemic treatment. A 65-year-old woman was diagnosed with WM 6 years ago and received Bruton tyrosine kinase inhibitor monotherapy for two years. Her INMZL was confirmed due to left cervical lymphadenopathy. The patient presented with oedema in both lower limbs one year ago, and was diagnosed with secondary light chain amyloidosis. Treatment with the BC regimen (rituximab 375 mg/m2 monthly for 6-8 courses, and bendamustine 90 mg/m2 per day × 2, monthly for six courses) was initiated, but not tolerated due to toxic side effects. Bortezomib-based therapy was given for two months, including bortezomib, dexamethasone, and zanubrutinb. Oedema in both lower limbs was relieved and treatment efficacy was evaluated as partial remission.
CONCLUSIONS: A detailed clinical evaluation and active identification of the aetiology are recommended to avoid missed diagnosis and misdiagnosis.
METHODS: We present a Chinese female patient who developed secondary light chain amyloidosis due to WM and INMZL and provides opinions on its systemic treatment. A 65-year-old woman was diagnosed with WM 6 years ago and received Bruton tyrosine kinase inhibitor monotherapy for two years. Her INMZL was confirmed due to left cervical lymphadenopathy. The patient presented with oedema in both lower limbs one year ago, and was diagnosed with secondary light chain amyloidosis. Treatment with the BC regimen (rituximab 375 mg/m2 monthly for 6-8 courses, and bendamustine 90 mg/m2 per day × 2, monthly for six courses) was initiated, but not tolerated due to toxic side effects. Bortezomib-based therapy was given for two months, including bortezomib, dexamethasone, and zanubrutinb. Oedema in both lower limbs was relieved and treatment efficacy was evaluated as partial remission.
CONCLUSIONS: A detailed clinical evaluation and active identification of the aetiology are recommended to avoid missed diagnosis and misdiagnosis.
摘要：
背景：Waldenström巨球蛋白血症（WM）与结间边缘区淋巴瘤（INMZL）并存很少，通常与预后不良有关。
方法：我们介绍了一例因WM和INMZL导致继发性轻链淀粉样变性的中国女性患者，并对其全身治疗提供了意见。一名65岁的女性在6年前被诊断为WM，并接受布鲁顿酪氨酸激酶抑制剂单药治疗两年。她的INMZL由于左颈部淋巴结肿大而被证实。病人一年前出现双下肢水肿,并被诊断为继发性轻链淀粉样变性。BC方案治疗（利妥昔单抗375mg/m2，每月治疗6-8个疗程，和苯达莫司汀90mg/m2/天×2，每月六个疗程）开始，但由于毒副作用而不能耐受。给予以硼替佐米为基础的治疗两个月，包括硼替佐米,地塞米松,还有Zanubrutinb.双下肢水肿缓解，治疗效果评估为部分缓解。
结论：建议进行详细的临床评估和积极的病因鉴定，以避免漏诊和误诊。
方法：我们介绍了一例因WM和INMZL导致继发性轻链淀粉样变性的中国女性患者，并对其全身治疗提供了意见。一名65岁的女性在6年前被诊断为WM，并接受布鲁顿酪氨酸激酶抑制剂单药治疗两年。她的INMZL由于左颈部淋巴结肿大而被证实。病人一年前出现双下肢水肿,并被诊断为继发性轻链淀粉样变性。BC方案治疗（利妥昔单抗375mg/m2，每月治疗6-8个疗程，和苯达莫司汀90mg/m2/天×2，每月六个疗程）开始，但由于毒副作用而不能耐受。给予以硼替佐米为基础的治疗两个月，包括硼替佐米,地塞米松,还有Zanubrutinb.双下肢水肿缓解，治疗效果评估为部分缓解。
结论：建议进行详细的临床评估和积极的病因鉴定，以避免漏诊和误诊。
