Tiu Andrew C, Potdar Rashmika, Arguello-Gerra Vivian, Morginstin Mark
Department of Medicine, Einstein Medical Center, Philadelphia, PA, USA.
Division of Hematology and Medical Oncology, Einstein Medical Center, Philadelphia, PA, USA.
Case Rep Hematol. 2018 May 24;2018:7954816. doi: 10.1155/2018/7954816. eCollection 2018.
Multiple myeloma is a malignant clonal proliferation of plasma cells in the bone marrow preceded by monoclonal gammopathy of undetermined significance. Initial presentation of multiple myeloma as extramedullary spread in soft tissues particularly in the liver is uncommon. We report a case of a 74-year-old African American female who presented with epigastric pain, hematemesis, elevated alkaline phosphatase, and gamma-glutamyl transferase. Initial impression was peptic ulcer disease; however, ultrasound and CT scan of the abdomen showed multiple liver nodules and perihepatic lymphadenopathy suggestive of metastatic disease. Biopsy of the liver nodules showed CD138 and kappa light chain-restricted positive cells consistent with extramedullary spread of multiple myeloma to the liver. The patient achieved partial response after 6 months of treatment with Velcade, cyclophosphamide, and dexamethasone (VCD). Due to severe neutropenia from cyclophosphamide, regimen was switched to Velcade, Revlimid, and dexamethasone (VRD) which resulted to very good partial response in 1 year which eventually persisted after 4 years. No controlled prospective studies have defined the standard treatment for multiple myeloma with extramedullary spread particularly to the liver. Treatment of multiple myeloma with extramedullary disease follows guidelines for multiple myeloma.
多发性骨髓瘤是骨髓中浆细胞的恶性克隆性增殖,之前常有意义未明的单克隆丙种球蛋白病。多发性骨髓瘤最初表现为软组织尤其是肝脏的髓外扩散并不常见。我们报告一例74岁非裔美国女性,她表现为上腹部疼痛、呕血、碱性磷酸酶和γ-谷氨酰转移酶升高。初步诊断为消化性溃疡疾病;然而,腹部超声和CT扫描显示肝脏有多个结节以及肝周淋巴结肿大,提示转移性疾病。肝脏结节活检显示CD138和κ轻链限制性阳性细胞,符合多发性骨髓瘤向肝脏的髓外扩散。患者在接受万珂、环磷酰胺和地塞米松(VCD)治疗6个月后获得部分缓解。由于环磷酰胺导致严重中性粒细胞减少,治疗方案改为万珂、来那度胺和地塞米松(VRD),1年后产生了非常好的部分缓解,最终在4年后仍持续缓解。尚无对照前瞻性研究明确多发性骨髓瘤髓外扩散尤其是扩散至肝脏的标准治疗方法。多发性骨髓瘤髓外疾病的治疗遵循多发性骨髓瘤的治疗指南。