Iosif Evangelia, Rees Clare, Beeslaar Salome, Shamali Awad, Lauro Roberto, Kyriakides Charis
Department of Gastrointestinal Surgery, Frimley Park Hospital, Frimley, Camberley GU16 7UJ, United Kingdom.
Department of Haematology, Frimley Park Hospital, Frimley, Camberley GU16 7UJ, United Kingdom.
World J Gastrointest Endosc. 2019 Apr 16;11(4):308-321. doi: 10.4253/wjge.v11.i4.308.
Plasma-cell neoplasms rarely involve the gastrointestinal tract and manifest as gastrointestinal bleeding. Plasmablastic myeloma is an aggressive plasma cell neoplasm associated with poor outcomes. A small number of cases with gastrointestinal involvement is reported in the literature and therefore high index of suspicion is essential for avoiding delays in diagnosis and treatment.
Our aim is to present our experience of a 70-year-old patient with a secondary presentation of plasmablastic myeloma manifesting as unstable upper gastrointestinal bleeding and to review the literature with the view to consolidate and discuss information about diagnosis and management of this rare entity. In addition to our case, a literature search (PubMed database) of case reports of extramedullary plasma cell neoplasms manifesting as upper gastrointestinal bleeding was performed. Twenty-seven cases of extramedullary plasmacytoma (EMP) involving the stomach and small bowel presenting with upper gastrointestinal bleeding were retrieved. The majority of patients were males (67%). The average age on diagnosis was 62.7 years. The most common site of presentation was the stomach (41%), followed by the duodenum (15%). The most common presenting complaint was melena (44%). In the majority of cases, the EMPs were a secondary manifestation (63%) at the background of multiple myeloma (26%), plasmablastic myeloma (7%) or high-grade plasma cell myeloma (4%). Oesophagogastroscopy was the main diagnostic modality and chemotherapy the preferred treatment option for secondary EMPs.
Despite their rare presentation, upper gastrointestinal EMPs should be considered in the differential diagnosis of patients with gastrointestinal bleeding especially in the presence of systemic haematological malignancy.
浆细胞肿瘤很少累及胃肠道，表现为胃肠道出血。浆母细胞性骨髓瘤是一种侵袭性浆细胞肿瘤，预后不良。文献报道了少数胃肠道受累的病例，因此高度怀疑对于避免诊断和治疗延误至关重要。
我们的目的是介绍一名70岁患者继发浆母细胞性骨髓瘤表现为上消化道出血不稳定的经验，并回顾文献以巩固和讨论关于这种罕见疾病的诊断和管理信息。除了我们的病例外，还对以消化道出血为表现的髓外浆细胞肿瘤的病例报告进行了文献检索（PubMed数据库）。检索到27例累及胃和小肠并表现为上消化道出血的髓外浆细胞瘤（EMP）。大多数患者为男性（67%）。诊断时的平均年龄为62.7岁。最常见的发病部位是胃（41%），其次是十二指肠（15%）。最常见的主诉是黑便（44%）。在大多数病例中，EMP是继发表现（63%），其背景为多发性骨髓瘤（26%）、浆母细胞性骨髓瘤（7%）或高级别浆细胞骨髓瘤（4%）。食管胃镜检查是主要的诊断方法，化疗是继发EMP的首选治疗选择。
尽管上消化道EMP表现罕见，但在胃肠道出血患者的鉴别诊断中应考虑，尤其是在存在全身性血液系统恶性肿瘤的情况下。
