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新发病例中单克隆型髓样肉瘤广泛累及胃肠道:一种常被误诊的罕见实体的病例报告。

Extensive involvement of the gastrointestinal tract by a de novo presentation of the monoblastic type of myeloid sarcoma: a case report of a rare entity that is often misdiagnosed.

机构信息

Department of Pathology & Laboratory Medicine, Medical University of South Carolina, Charleston, South Carolina 29425-2900, USA.

出版信息

Am J Med Sci. 2009 Dec;338(6):513-6. doi: 10.1097/MAJ.0b013e3181b26fa9.

Abstract

We present the first known case of the monoblastic type of myeloid sarcoma (also known as extramedullary myeloid tumor, chloroma, and granulocytic sarcoma) with diffuse involvement of the gastrointestinal tract. The patient originally presented with diarrhea and crampy abdominal discomfort. Endoscopically, the disease showed a diffuse inflammatory process mimicking a number of benign conditions, such as inflammatory bowel disease and autoimmune enteropathy. Sequential biopsies of the upper and lower gastrointestinal tract showed a diffuse infiltrate of increasingly atypical cells. The disease progressed to systemic involvement, including widespread lymphadenopathy, splenomegaly, and pulmonary deposits; the patient died 13 months after the development of initial symptoms. The immunohistochemical and histologic profiles of this case are diagnostic of the monoblastic type of myeloid sarcoma.

摘要

我们呈现首例弥漫累及胃肠道的原始单核细胞型髓外髓系肉瘤(也称为髓外髓样肿瘤、绿色瘤和粒细胞肉瘤)。患者最初表现为腹泻和痉挛性腹痛。内镜下,该疾病表现为弥漫性炎症过程,类似于多种良性疾病,如炎症性肠病和自身免疫性肠炎。上消化道和下消化道的连续活检显示弥漫性浸润的细胞逐渐变得非典型。疾病进展为全身性受累,包括广泛的淋巴结病、脾肿大和肺部沉积;患者在初始症状出现后 13 个月死亡。该病例的免疫组织化学和组织学特征诊断为原始单核细胞型髓外髓系肉瘤。

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