Spier Bret J, Einstein Michael, Johnson Eric A, Zuricik Andrew O, Hu Johnny L, Pfau Patrick R
Gastroenterology and Hepatology, Department of Internal Medicine, University of Wisconsin School of Medicine and Public Health, Madison, WI 53792-5124, USA.
WMJ. 2008 Feb;107(1):40-3.
AL-Amyloid rarely presents in the gastrointestinal tract as acute gastrointestinal hemorrhage, especially in the absence of clinical disease elsewhere in the body. There are no reported cases of monoclonal gammopathy of undetermined significance progressing to AL-Amyloid presenting as lower gastrointestinal hemorrhage. We report a case of a patient initially diagnosed with monoclonal gammopathy of undetermined significance who progressed to AL-Amyloid over the course of 1 year. His progression resulted in primary colonic amyloidosis that manifested as lower gastrointestinal hemorrhage. The diagnosis was made by biopsy of a sigmoid plaque demonstrating necrotic material on histopathology. Amyloid deposition was seen on congo red and on birefringence. The bleeding stopped spontaneously without intervention and he was discharged his fourth day in the hospital. Further evaluation revealed no involvement in other organ systems. The plan is to treat with melphalan and dexamethasone. We conclude that early endoscopic examination and biopsy of the surrounding intestinal tissue is indicated when patients with monoclonal gammopathy of undetermined significance present with gastrointestinal hemorrhage to evaluate for the progression to AL-Amyloidosis. Treatment to prevent recurrent hemorrhage and further progression of the disease should be considered.
淀粉样轻链(AL)淀粉样变很少以急性胃肠道出血的形式出现在胃肠道,尤其是在身体其他部位无临床疾病的情况下。目前尚无意义未明的单克隆丙种球蛋白病进展为以胃肠道下消化道出血为表现的AL淀粉样变的报道病例。我们报告一例患者,最初被诊断为意义未明的单克隆丙种球蛋白病,在1年的病程中进展为AL淀粉样变。其病情进展导致原发性结肠淀粉样变,表现为下消化道出血。通过对乙状结肠斑块进行活检确诊,组织病理学显示有坏死物质。刚果红染色及双折射检查可见淀粉样沉积。出血未经干预自行停止,患者于住院第四天出院。进一步评估显示其他器官系统未受累。计划采用美法仑和地塞米松进行治疗。我们得出结论,当意义未明的单克隆丙种球蛋白病患者出现胃肠道出血时,应尽早进行内镜检查并对周围肠道组织进行活检,以评估是否进展为AL淀粉样变性。应考虑采取治疗措施以预防出血复发和疾病的进一步进展。