Ariyarathenam Arun, Galvin Nick, Akoh Jacob A
Gastroenterology, Surgery & Renal Services Directorate, Plymouth Hospitals NHS Trust, Derriford Hospital, Plymouth PL6 8DH, United Kingdom.
Int J Surg Case Rep. 2013;4(5):486-8. doi: 10.1016/j.ijscr.2013.02.018. Epub 2013 Mar 14.
Multiple myeloma is a monoclonal, immunoproliferative plasma-cell neoplasm of the B lymphoid cells. Extramedullary plasmacytoma is a type of plasma-cell neoplasm that can present as a primary tumour or secondary to another plasma-cell neoplasm, such as multiple myeloma. Secondary extramedullary plasmacytoma is usually noted in the advanced stages of the disease with ileum involvement being very rare.
We report a rare case of a 58-year-old man, with known multiple myeloma, re-presenting with evidence of small bowel obstruction, secondary to an intussusception due to a malignant plasma cell deposit, which was successfully resected at laparotomy. Previous two similar admissions, prior to this index admission, failed to arrive at this difficult rare diagnosis.
Primary and secondary extramedullary plasmacytoma mainly affects the upper aero-digestive tract. Involvement of the ileum, as in this case, is a rare complication. Prognosis of secondary extramedullary plasmacytoma affecting the gastrointestinal tracts is unknown, due to the small number of cases reported in the literature, but suggestive of a poor prognosis. The role of surgery is often palliative to deal with resolvable life-threatening emergencies and where possible to prolong life.
The case adds to the current literature of the rare event of visceral secondary extramedullary plasmacytoma involving the gastrointestinal tract, in the course of multiple myeloma and highlights the need for a high index of suspicion for such uncommon complications, to avoid delay in diagnosis and treatment.
多发性骨髓瘤是一种B淋巴细胞的单克隆免疫增殖性浆细胞瘤。髓外浆细胞瘤是浆细胞瘤的一种类型,可表现为原发性肿瘤或继发于另一种浆细胞瘤,如多发性骨髓瘤。继发性髓外浆细胞瘤通常在疾病晚期出现,累及回肠的情况非常罕见。
我们报告一例罕见病例,一名58岁男性,已知患有多发性骨髓瘤,因恶性浆细胞沉积导致肠套叠继发小肠梗阻再次就诊,在剖腹手术中成功切除。在本次索引入院之前的两次类似入院均未能做出这一困难的罕见诊断。
原发性和继发性髓外浆细胞瘤主要影响上呼吸道消化道。如本病例中累及回肠是一种罕见的并发症。由于文献报道的病例数量较少,继发性髓外浆细胞瘤累及胃肠道的预后尚不清楚,但提示预后不良。手术的作用通常是姑息性的,以处理可解决的危及生命的紧急情况,并在可能的情况下延长生命。
该病例增加了目前关于多发性骨髓瘤病程中累及胃肠道的内脏继发性髓外浆细胞瘤这一罕见事件的文献,并强调对此类罕见并发症需要高度怀疑,以避免诊断和治疗延误。