Staderini Michela, Mannelli Lara, Antonioli Elisabetta, Puccini Benedetta, Berti Valentina, Mungai Francesco, Vergoni Federica, Carrai Valentina, Rigacci Luigi, Bosi Alberto
Haematology Unit, Careggi University Hospital, Florence, Italy.
Medical Genetics, University of Siena, Siena, Italy.
Case Rep Hematol. 2020 Jan 16;2020:6309736. doi: 10.1155/2020/6309736. eCollection 2020.
ALK-negative anaplastic large cell lymphoma is a rare T-cell neoplasm with an aggressive course requiring prompt diagnostic work-up and treatment. Few cases of concomitant multiple myeloma and T-cell neoplasm are described in the literature, mainly regarding primary cutaneous anaplastic large cell lymphoma. We present the case of a 65-year-old man, simultaneously diagnosed with ALK-negative anaplastic large cell lymphoma with extranodal localization in the gastrocnemius muscle (stage 1AE) and IgG lambda multiple myeloma (ISS 2, Durie-Salmon stage 3A). Both diseases required therapeutic intervention due to the high proliferative index of lymphoma and the presence of bone lesions attributable to myeloma. The therapeutic program initially included chemotherapy (cyclophosphamide, doxorubicin, vincristine, etoposide, and prednisone; CHOEP), radiotherapy on the leg, bortezomib, and then consolidation with autologous hematopoietic stem cell transplantation. Despite being on bortezomib treatment and waiting for transplantation, the patient experienced an early myeloma progression that turned out to be refractory to second-line lenalidomide-based treatment. To our knowledge, this is the first case of concurrent diagnosis of extranodal ALK-negative anaplastic large cell lymphoma of the muscle and multiple myeloma. Simultaneous onset can be challenging for clinicians as both diseases may have an aggressive course requiring multiple treatments with increased risk of toxicity and complicated management.
ALK阴性间变性大细胞淋巴瘤是一种罕见的T细胞肿瘤，病程侵袭性强，需要及时进行诊断检查和治疗。文献中很少描述同时合并多发性骨髓瘤和T细胞肿瘤的病例，主要是关于原发性皮肤间变性大细胞淋巴瘤。我们报告一例65岁男性患者，同时被诊断为ALK阴性间变性大细胞淋巴瘤，结外定位在腓肠肌（1AE期）和IgG λ型多发性骨髓瘤（国际分期系统2期，Durie-Salmon分期3A期）。由于淋巴瘤的高增殖指数以及骨髓瘤所致的骨病变，两种疾病均需要进行治疗干预。治疗方案最初包括化疗（环磷酰胺、阿霉素、长春新碱、依托泊苷和泼尼松；CHOEP方案）、腿部放疗、硼替佐米，然后进行自体造血干细胞移植巩固治疗。尽管患者接受了硼替佐米治疗并等待移植，但仍出现早期骨髓瘤进展，结果显示对基于来那度胺的二线治疗无效。据我们所知，这是首例同时诊断肌肉型结外ALK阴性间变性大细胞淋巴瘤和多发性骨髓瘤的病例。同时发病对临床医生来说可能具有挑战性，因为两种疾病都可能病程侵袭性强，需要多次治疗，毒性风险增加且管理复杂。
