Department of Internal Medicine I (Gastroenterology, Hepatology, Endocrinology and Metabolism), Medical University of Innsbruck, Innsbruck, Austria.
Department of Visceral, Transplant and Thoracic Surgery, Medical University of Innsbruck, Innsbruck, Austria.
J Med Case Rep. 2020 Oct 25;14(1):201. doi: 10.1186/s13256-020-02511-9.
The involvement of vital organs in multiple myeloma (MM) with systemic amyloid light-chain (AL) amyloidosis can lead to acute organ failure. In this case, the fear of recurrence or progression of multiple myeloma often excludes those patients from undergoing organ transplantation. Nevertheless, clinically fit patients might benefit from a different therapeutic approach. This case presentation might highlight this particular unmet need and strengthen a different treatment approach.
To our knowledge, we present the first case of successful simultaneous liver and kidney transplantation, followed by autologous stem cell transplantation in a 60-year-old Caucasian male patient suffering from MM (Durie-Salmon stage IIB; ISS-stage: III, RISS stage: III) with primary AL amyloidosis. Chemotherapy treatment led to end-stage kidney disease requiring dialysis. Liver failure also occurred after at least three cycles of CyBorD (bortezomib, cyclophosphamide, and dexamethasone) of induction therapy with a good hematologic response. Over three years after the initial diagnosis, the patient is reportedly showing an excellent quality of life and a complete remission.
We conclude that kidney and liver transplantation followed by autologous stem cell transplantation can be a treatment option for a selected group of patients with MM if AL amyloidosis is leading. In the end, the remission assessment by IMWG response criteria displayed a complete remission of MM together with complete reconstitution of organ functions (liver & renal function) as long as upfront clinical evaluation excludes significant cardiac involvement and other severe co-morbidities.
多发性骨髓瘤（MM）合并系统性轻链（AL）淀粉样变性可累及重要器官，导致急性器官衰竭。在这种情况下，由于担心多发性骨髓瘤复发或进展，往往排除这些患者进行器官移植。然而，临床情况适合的患者可能会受益于不同的治疗方法。本病例报告可能强调了这一未满足的需求，并强化了不同的治疗方法。
据我们所知，我们报告了首例成功同时进行肝、肾移植，随后进行自体干细胞移植的病例，该患者为 60 岁白人男性，患有 MM（Durie-Salmon 分期 IIB；ISS 分期：III，RISS 分期：III）合并原发性 AL 淀粉样变性。化疗导致终末期肾病需要透析。在至少三个周期的 CyBorD（硼替佐米、环磷酰胺和地塞米松）诱导治疗后，肝衰竭也发生了，诱导治疗有良好的血液学反应。在初始诊断后的三年多时间里，据报道该患者生活质量良好，完全缓解。
我们得出结论，如果 AL 淀粉样变性是主要原因，那么肾、肝移植后进行自体干细胞移植可以作为 MM 患者的一种治疗选择。最终，根据 IMWG 缓解标准评估，多发性骨髓瘤完全缓解，同时肝肾功能完全恢复正常，只要初始临床评估排除严重的心脏受累和其他严重合并症。
