Piotr Jachimowski , Łukasz Ciulkiewicz , Mateusz Ziarkiewicz , Bogna Ziarkiewicz-Wróblewska , Marta Legatowicz-Koprowska , Krzysztof Jamroziak
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引用次数: 0
摘要
瓦尔登斯特伦巨球蛋白血症（WM）是一种罕见的非霍奇金B细胞淋巴瘤，具有淋巴浆细胞形态，伴有免疫球蛋白M（IgM）单克隆性腺病。WM与免疫球蛋白轻链（AL）淀粉样变性同时存在，这种现象并不常见，但已被详细描述。与非 IgM 患者相比，IgM AL 淀粉样变性患者的软组织受累和神经病变更为普遍。我们报告了一例82岁的女性患者，她患有WM和并发症IgM AL淀粉样变性，双下肢出现大量淀粉样变瘤，但没有明显的心脏和肾脏受累。患者对多种治疗方法均无效，最后开始接受扎鲁替尼单药治疗，并获得了快速、持续的非常好的部分血液学反应（VGPR）和临床改善。
Refractory IgM AL amyloidosis with massive soft tissue tumors: Rescue with zanubrutinib. A case report
Waldenström macroglobulinemia (WM) is a rare indolent B-cell non-Hodgkin lymphoma with lymphoplasmacytic morphology, associated with immunoglobulin M (IgM) monoclonal gammopathy. The coexistence of WM and immunoglobulin light chain (AL) amyloidosis is an uncommon but well-described phenomenon. In patients suffering from IgM AL amyloidosis soft tissue involvement and neuropathy are more prevalent in comparison to non-IgM patients. We present a case of 82-year old female with WM and intercurrent IgM AL amyloidosis, presenting with massive amyloidomas of both lower extremities, without significant cardiac and renal involvement. The patient was refractory to several lines of treatment and finally started on zanubrutinib monotherapy, with rapid and sustained very good partial hematologic response (VGPR) and clinical improvement.
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