Published online Apr 16, 2019. doi: 10.4253/wjge.v11.i4.308
Peer-review started: February 17, 2019
First decision: March 11, 2019
Revised: March 27, 2019
Accepted: April 8, 2019
Article in press: April 9, 2019
Published online: April 16, 2019
Processing time: 60 Days and 11.1 Hours
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.
Core tip: The involvement of gastrointestinal tract by plasma cell neoplasms and the manifestation as gastrointestinal bleeding is very rare. However, patients can be profoundly unstable on presentation requiring immediate diagnosis and intervention. As the existing literature is very scattered and it mainly consists of case reports, we are aiming with our present work not only to describe our experience but also to review, consolidate and discuss information about diagnosis and management of this rare cause of gastrointestinal bleeding. The management these patients requires a multidisciplinary team approach and should involve not only the gastroenterology and surgical teams but also haematology and oncology teams for achievement of the best possible outcomes.