Liu Ai-Ling, Ding Xue-Li, Liu Hua, Zhao Wen-Jun, Jing Xue, Zhou Xuan, Mao Tao, Tian Zi-Bin, Wu Jun
Department of Gastroenterology, The Affiliated Hospital of Qingdao University, Qingdao 266003, Shandong Province, China.
Department of Pathology, The Affiliated Hospital of Qingdao University, Qingdao 266003, Shandong Province, China.
World J Clin Cases. 2022 Mar 6;10(7):2307-2314. doi: 10.12998/wjcc.v10.i7.2307.
Smoldering multiple myeloma (SMM) is an asymptomatic plasma cell proliferative disorder that can progress to multiple myeloma (MM). Amyloidosis (light chain) (AL) is the most common form of systemic amyloidosis. There are few reports of SMM coexisting with AL involving the digestive tract.
A 63-year-old woman presented with lower limb edema, abdominal distension, abdominal pain, and hematochezia. Gastroscopy showed gastric retention, gastric angler mucosal coarseness, hyperemia, and mild oozing of blood. Colonoscopy showed hyperemic and edematous mucosa of the distal ascending colon and sigmoid colon with the presence of multiple round and irregular ulcers, submucosal ecchymosis, and hematoma. Gastric and colonic tissue biopsy confirmed the diagnosis of AL by positive Congo red staining. MM was confirmed by bone marrow biopsy and immunohistochemistry. The patient had no hypercalcemia, renal dysfunction, anemia, bone lesions or biomarkers of malignancy defined as plasma cells > 60% in bone marrow. Additionally, no elevated serum free light chain ratio, or presence of bone marrow lesions by magnetic resonance imaging (SLiM criteria) were detected. The patient was finally diagnosed with SMM coexisting with AL. She received chemotherapy and was discharged when the symptoms were relieved. She is doing well at nearly five years of follow up.
This case highlights that high index of suspicion is required to diagnose gastrointestinal AL. It should be suspected in elderly patients with endoscopic findings of granular-appearing mucosa, ecchymosis, and submucosal hematoma. Timely diagnosis and appropriate therapy can help to improve the prognosis of these patients.
冒烟型多发性骨髓瘤（SMM）是一种无症状的浆细胞增殖性疾病，可进展为多发性骨髓瘤（MM）。淀粉样变性（轻链型）（AL）是系统性淀粉样变性最常见的形式。SMM与累及消化道的AL共存的报道较少。
一名63岁女性出现下肢水肿、腹胀、腹痛和便血。胃镜检查显示胃潴留、胃角黏膜粗糙、充血及轻度渗血。结肠镜检查显示升结肠远端和乙状结肠黏膜充血水肿，有多个圆形及不规则溃疡、黏膜下瘀斑和血肿。胃和结肠组织活检刚果红染色阳性确诊为AL。骨髓活检和免疫组化确诊为MM。患者无高钙血症、肾功能不全、贫血、骨病变或定义为骨髓中浆细胞>60%的恶性生物标志物。此外，未检测到血清游离轻链比值升高或磁共振成像显示的骨髓病变（SLiM标准）。该患者最终被诊断为SMM合并AL。她接受了化疗，症状缓解后出院。近5年随访情况良好。
该病例强调诊断胃肠道AL需要高度怀疑。对于内镜检查发现黏膜呈颗粒状、瘀斑和黏膜下血肿的老年患者应予以怀疑。及时诊断和适当治疗有助于改善这些患者的预后。
