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致命性胃肠道出血作为骨髓增生异常综合征患者粒细胞肉瘤的主要表现

Fatal gastrointestinal bleeding as the primary manifestation of granulocytic sarcoma in a patient with myelodysblastic syndrome.

作者信息

Saleh H A, Khatib G

机构信息

Department of Pathology, Grace Hospital, USA.

出版信息

J Fla Med Assoc. 1997 Feb;84(2):111-4.

PMID:9066237
Abstract

Granulocytic sarcoma (GS), or chloroma, is a rare extramedullary tumor composed of immature myeloid cells. It most commonly involves bone, soft tissue, lymph nodes and skin and develops during the course of or preceding myelogenous leukemia (ML). Involvement of other organs has been rarely reported including ovary, uterus and cervix, lung and the gastrointestinal tract; however, GS presenting as upper and lower gastrointestinal (GI) bleeding from ulcerated gastric mass and concurrent bleeding vaginal mass is an unusual rare manifestation of GS. We describe a case of GS in a 70 year old black woman who presented with a bleeding "lump" in the vaginal wall and suffered fatal GI bleeding from an ulcerated gastric lesion. She was diagnosed with myelodysblastic syndrome a few months earlier. From the review of the available English literature, this is a unique presentation of GS. It is important to include this entity in the differential diagnosis when encountering GI bleeding particularly in a patient previously diagnosed with myeloid leukemia or preleukemia. The importance of Naphthol Chloracetate Esterase (NCAE) stain and lysozyme immunoperoxidase stain in establishing the diagnosis is breifly discussed.

摘要

粒细胞肉瘤(GS),又称绿色瘤,是一种由未成熟髓样细胞组成的罕见髓外肿瘤。它最常累及骨骼、软组织、淋巴结和皮肤,在骨髓性白血病(ML)病程中或之前发生。其他器官受累的情况鲜有报道,包括卵巢、子宫和宫颈、肺及胃肠道;然而,GS表现为溃疡性胃肿块导致的上、下消化道(GI)出血以及同时出现的阴道肿块出血是GS一种不寻常的罕见表现。我们报道一例70岁黑人女性的GS病例,她表现为阴道壁有出血性“肿块”,并因溃疡性胃部病变导致致命的消化道出血。几个月前她被诊断为骨髓增生异常综合征。通过检索现有的英文文献,这是GS的一种独特表现。在遇到消化道出血时,尤其是先前被诊断为髓样白血病或白血病前期的患者,将该疾病纳入鉴别诊断很重要。本文简要讨论了醋酸萘酯酶(NCAE)染色和溶菌酶免疫过氧化物酶染色在确诊中的重要性。

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Fatal gastrointestinal bleeding as the primary manifestation of granulocytic sarcoma in a patient with myelodysblastic syndrome.致命性胃肠道出血作为骨髓增生异常综合征患者粒细胞肉瘤的主要表现
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