多发性骨髓瘤累及胰腺患者的临床分析

Clinical analysis of patients with multiple myelomna involving the pancreas

目的:探讨多发性骨髓瘤(MM)累及胰腺的流行病学、发病机制、临床表现、治疗及预后。方法:回顾分析2011年10月至2021年10月收治台州医院的4例MM累及胰腺患者的临床表现、治疗及转归情况,并复习相关文献。结果:4例患者,男3例,女1例,年龄53~64岁,3例IgG/λ型,1例IgG/κ型;DS分期IA期2例、IIIA期2例;ISS分期II期2例、III期2例;累及胰腺距离MM确诊时间为16.3~92.2个月,4例初诊时均无髓外受累(EMD),但累及胰腺前或时均存在其他部位的EMD。累及胰腺时3例存在乏力、眼黄、腰酸;3例血淀粉酶升高,2例胆红素升高,2例CA199升高。4例均累及胰头、2例同时累及胰尾、1例还累及胰体;胰腺病灶在经腹B超下易漏诊,CT平扫呈稍低密度,增强后多轻度强化,PET/CT中病灶标准摄取值(SUV)升高。2例骨髓和EMD(胸腹水、胰腺)中的CD56表达均明显下调。确诊后给予蛋白酶体抑制剂联合来那度胺或者蒽环类/铂类药物化疗,获得了短期的疗效,胰腺累及确诊后总生存仅1.7~7.8个月,其中3例死于严重感染。结论:MM继发胰腺累及极为罕见,可在MM的不同阶段出现,常伴随或者晚于其他部位的EMD出现,一旦出现往往意味着疾病终末期,CD56表达下调可能参与其发生发展,目前尚无有效的治疗手段,新型药物的联合治疗或可改善疗效。

Objective:To investigate the epidemiology,pathogenesis,clinical manifestations,treatment and prognosis of multiple myeloma(MM)involving the pancreas.Methods:We retrospectively analyzed the clinical manifestations,treatment and prognosis of 4 patients with MM involving the pancreas admitted to Taizhou Hospital,Zhejiang Province,from October 2011 to October 2021,and reviewed the relevant literature.Results:Four patients,3 male and 2 female aged 53 to 64,of whom 3 were IgG/λtype and 1 was IgG/κtype;2 were IA and 2 IIIA(DS staging system);2 were II and 2 were III(ISS staging system).The interval between the diagnosis of MM involving the pancreas and MM was 16.3~92.2 months.None of the four cases had extramedullary disease(EMD)at the time of initial diagnosis of MM,but had EMD at other sites before or at the time of pancreatic involvement.Three cases had fatigue,icteric sclera,and lumbago at the time of pancreatic involvement,and one case had no discomfort;three cases had elevated amylase,and 2 had elevated bilirubin;CA199 was elevated in 2 cases.The head of the pancreas was involved in all four cases;the tail was involved in 2 cases and the pancreatic body in one case;the pancreatic lesions were easy to be missed on ultrasound through the abdomen,and the lesions appeared to be slightly hypointense on CT plain scan,mildly intensified after enhancement,and had an elevated SUV value on PET/CT.In 2 cases,the expression of CD56 was significantly downregulated in both the bone marrow and the EMD(thoracic and abdominal fluid,pancreas).Chemotherapy with proteasome inhibitors combined with lenalidomide or anthracycline/platinum agents was given after diagnosis,which achieved short-term efficacy.Overall survival was only 1.7-7.8 months after diagnosis of pancreatic involvement,and 3 of them died of severe infections.Conclusion:Pancreatic involvement secondary to MM is rare,which occurs at different stages of MM and is often accompanied with or later than other EMD.Its presence often suggests the end-stage of MM.The decreased expression of CD56 may be related to its development.Without effective treatment currently,the efficacy might be improved by the combination of novel drugs.