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一例以胃肠道出血和伊文氏综合征为表现的多发性骨髓瘤病例。

A Case of Multiple Myeloma Presenting with Gastrointestinal Bleeding and Evans Syndrome.

作者信息

Khan Muhammad S, Kasmani Rahil, Khan Ghazal, Changal Khalid, Singh Hemindermeet

机构信息

Internal Medicine, Mercy St. Vincent Medical Center, Toledo, USA.

Nephrology, Mercy St. Vincent Medical Centre, Toledo, USA.

出版信息

Cureus. 2019 Oct 22;11(10):e5969. doi: 10.7759/cureus.5969.

Abstract

Autoimmune events are rare in multiple myeloma (MM). Herein, we report a rare case of a patient presenting with recurrent gastrointestinal (GI) bleeding of unknown origin, also having pancytopenia eventually diagnosed as MM with Evans syndrome. This is an uncommon disorder presenting as autoimmune hemolytic anemia (AIHA) with immune thrombocytopenia purpura (ITP). A 56-year-old African American male presenting with recurrent GI bleeds and pancytopenia of unknown origin developed acute colonic diverticulitis on recurrent admissions, and sigmoid colectomy with primary anastomosis was performed. Flow cytometry with serum protein electrophoresis eventually revealed IgG MM with elevated Kappa/Lambda ratio. Bone marrow biopsy revealed 80% to 90% Kappa clonal plasma cells confirming MM. Direct antiglobulin test (DAT) was positive with pancytopenia. The patient initially showed a good response to chemotherapy with thrombocytopenia improving with intravenous (I/V) dexamethasone. DAT done after completion of initial chemotherapy was negative. However, his disease relapsed after three months with pancytopenia and DAT becoming positive again. The patient was restarted on chemotherapy for debulking, which resulted in a negative DAT again after two months, but pancytopenia did not improve. The patient eventually passed away due to subarachnoid hemorrhage. We highlight only this fourth reported case because of its unique presentation. In elderly patients with unknown cause of GI bleeding with pancytopenia, blood dyscrasias, especially MM, should be considered. Autoimmune workup if positive might warrant the use of steroids for pancytopenia, which can improve thrombocytopenia in MM with Evans syndrome but not anemia.

摘要

自身免疫事件在多发性骨髓瘤(MM)中较为罕见。在此,我们报告一例罕见病例,患者反复出现不明原因的胃肠道(GI)出血,同时伴有全血细胞减少,最终被诊断为伴有Evans综合征的MM。这是一种罕见的疾病,表现为自身免疫性溶血性贫血(AIHA)合并免疫性血小板减少性紫癜(ITP)。一名56岁的非裔美国男性,反复出现不明原因的GI出血和全血细胞减少,在反复入院时发生急性结肠憩室炎,并进行了乙状结肠切除术及一期吻合术。流式细胞术和血清蛋白电泳最终显示为IgG型MM,κ/λ比值升高。骨髓活检显示80%至90%的κ克隆浆细胞,确诊为MM。直接抗球蛋白试验(DAT)呈阳性且伴有全血细胞减少。患者最初对化疗反应良好,血小板减少症通过静脉注射(I/V)地塞米松得到改善。初始化疗完成后进行的DAT为阴性。然而,三个月后疾病复发,全血细胞减少,DAT再次变为阳性。患者重新开始化疗以减瘤,两个月后DAT再次转为阴性,但全血细胞减少症并未改善。患者最终因蛛网膜下腔出血去世。由于其独特的表现,我们仅强调这第四例报告病例。对于不明原因的GI出血合并全血细胞减少的老年患者,应考虑血液系统疾病,尤其是MM。自身免疫检查如果呈阳性,可能有必要使用类固醇治疗全血细胞减少症,这可以改善伴有Evans综合征的MM患者的血小板减少症,但对贫血无效。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d51e/6867356/fd3153f1cf25/cureus-0011-00000005969-i01.jpg

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