Duodenal Ulcer with Massive Gastrointestinal Hemorrhage as an Initial Manifestation in Multiple Myeloma with Extramedullary Disease: A Case Report

Abstract

Plasma cell neoplasms are characterized by dysregulated proliferation of mature B cells, which can present with either single (solitary plasmacytoma) or systemic (multiple myeloma (MM)) involvement. MM with extramedullary plasmacytoma (EMP) is a rare disease that accounts for approximately 3–5% of all plasmacytomas. EMP with gastrointestinal (GI) system involvement is an even rarer entity, accounting for <1% of MM cases. We present a case of aggressive MM with EMP invading the duodenum, initially presented with massive upper GI hemorrhage and small bowel obstruction. A 67-year-old woman was admitted to our hospital owing to a lack of either gas or feces passage for 3 days. Abdominal distention and vomit with a high coffee ground content were observed for 24 h. The patient’s condition was initially diagnosed as small bowel obstruction, upper gastrointestinal bleeding, severe anemia, acute renal failure, and hypercalcemia. Furthermore, an analysis of immunoelectrophoresis in the blood, bone marrow aspiration, and tissue biopsy supported the diagnosis of MM and EMP invading the duodenum, upper GI hemorrhage, and small bowel obstruction. Our study provided the possible involvement of MM and EMP in the differential diagnosis of patients with unexplained GI hemorrhage and small bowel obstruction. A thorough review of the literature regarding the association between MM, GI hemorrhage, and small bowel obstruction is presented in this study.

Gastrointestinal, hepatic, and pancreatobiliary involvement by plasma cell neoplasms: clinicopathologic correlations in a retrospective cohort of 116 cases

“…Second, the distinction between a true PC and EMM with marrow involvement is crucial, as management and prognosis may differ markedly. 8,[12][13][14][15][16]18,28 For example, radiotherapy is typically first-line therapy for PC, [29][30][31][32][33][34] while chemotherapy and HSCT are typically employed for treating EMM. 29,33 In addition, progression of marrow disease to EMM may alter management, such as the addition of anthracyclinecontaining combination chemotherapy.…”

“…First, EMM may be present before any disease is found in the marrow, and thus may be the initial finding in a patient with MM. Second, the distinction between a true PC and EMM with marrow involvement is crucial, as management and prognosis may differ markedly 8,12–16,18,28 . For example, radiotherapy is typically first‐line therapy for PC, 29–34 while chemotherapy and HSCT are typically employed for treating EMM 29,33 .…”

“…15 The described signs and symptoms of GI PC and EMM vary widely and range from asymptomatic patients to those with fatigue, abdominal pain, GI bleeding, amyloidosis, organomegaly, polyps, ulcers, or obstructing masses that simulate carcinomas. [16][17][18][19][20][21][22][23][24] Interestingly, the available data suggest that patients with PC have a worse prognosis than those with EMM, often with relapsing disease or marrow involvement that can occur years after surgical resection. 16 As most of the reports of GI involvement by PCNs are in the form of case reports or small case series, we performed a multi-institutional study to describe the clinicopathologic features of GI PCNs in detail.…”

“…GI PCs are even less common, but have been rarely reported in the stomach, 12 small intestine, 13,14 colon, 12 and retroperitoneum 15 . The described signs and symptoms of GI PC and EMM vary widely and range from asymptomatic patients to those with fatigue, abdominal pain, GI bleeding, amyloidosis, organomegaly, polyps, ulcers, or obstructing masses that simulate carcinomas 16–24 . Interestingly, the available data suggest that patients with PC have a worse prognosis than those with EMM, often with relapsing disease or marrow involvement that can occur years after surgical resection 16 …”