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胰腺Rosai-Dorfman病(伴巨大淋巴结病的窦组织细胞增生症):首例病例报告

Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) of the pancreas: first case report.

作者信息

Esquivel J, Krishnan J, Jundi M, Sugarbaker P H

机构信息

Department of Surgical Oncology, Washington Cancer Institute, Washington, DC, USA.

出版信息

Hepatogastroenterology. 1999 Mar-Apr;46(26):1202-5.

PMID:10370692
Abstract

Rosai-Dorfman Disease is a histiocytic proliferative disorder which primarily affects lymph nodes. Extranodal involvement occurs in about one half of the patients and the head and neck area represents the region most commonly involved. We present the case of a 48 year-old female who was found with a pancreatic mass during evaluation for abdominal pain. She underwent a distal pancreatectomy and splenectomy. Her pathology showed sinus histiocytosis with massive lymphadenopathy (SHML) involving the pancreas and lymph nodes and focally the spleen. The histiocytes characteristically contained one or more viable lymphocytes in the cytoplasm. The lymphocytes had penetrated the cytoplasm in a process known as "emperipolesis", where the lymphocytes continued to have free movement in the histiocyte. The histiocytic cells were positive with S-100 protein and CD68. Rosai-Dorfman Disease (SHML) can affect the peripancreatic lymph nodes with possible secondary pancreatic involvement and present as a pancreatic mass.

摘要

罗萨伊-多夫曼病是一种主要累及淋巴结的组织细胞增生性疾病。约一半的患者会出现结外受累,头颈部是最常受累的区域。我们报告一例48岁女性病例,该患者在因腹痛接受评估时发现胰腺肿块。她接受了远端胰腺切除术和脾切除术。其病理显示为窦组织细胞增生伴巨大淋巴结病(SHML),累及胰腺、淋巴结,脾脏局部受累。组织细胞的特征是细胞质内含有一个或多个活淋巴细胞。淋巴细胞通过一种称为“血细胞穿入现象”的过程穿透细胞质,在此过程中淋巴细胞在组织细胞内仍可自由移动。组织细胞对S-100蛋白和CD68呈阳性反应。罗萨伊-多夫曼病(SHML)可累及胰腺周围淋巴结,可能继发胰腺受累,并表现为胰腺肿块。

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Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) of the pancreas: first case report.胰腺Rosai-Dorfman病(伴巨大淋巴结病的窦组织细胞增生症):首例病例报告
Hepatogastroenterology. 1999 Mar-Apr;46(26):1202-5.
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Development of disseminated histiocytic sarcoma in a patient with autoimmune lymphoproliferative syndrome and associated Rosai-Dorfman disease.自身免疫性淋巴组织增生综合征伴 Rosai-Dorfman 病患者发生播散性组织细胞肉瘤。
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