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罗萨伊-多夫曼病——两例伴有淋巴结及结外受累的罕见病例报告

Rosai Dorfman disease--a rare entity report of two cases with nodal and extranodal involvement.

作者信息

Jalil Jawad, Dar Nasser Rashid, Shafique Mobeen

机构信息

Department of Paediatrics, Combined Military Hospital, Bahawalpur.

出版信息

J Pak Med Assoc. 2008 Jun;58(6):338-40.

Abstract

Rosai Dorfman Disease (RDD) also known as Sinus Histiocytosis with Massive Lymphadenopathy (SHML)is a very rare variety of reactive histiocytoses. It commonly involves cervical lymph nodes; although involvement of other lymph node regions, skin and other organ involvement can occur. It has a good prognosis so there is a need to differentiate it from other lympho proliferative disorders of poorer prognosis. We present two cases of this rare disorder illustrating its clinical spectrum; a 22 years old woman with involvement of submandibular lymph nodes, nasal septum and sub-glottic region and a 45 years old male with prominent skin involvement.

摘要

罗萨伊-多夫曼病(RDD),也称为伴有巨大淋巴结病的窦性组织细胞增生症(SHML),是一种非常罕见的反应性组织细胞增生症。它通常累及颈部淋巴结;尽管其他淋巴结区域、皮肤及其他器官也可能受累。其预后良好,因此有必要将其与其他预后较差的淋巴增生性疾病相鉴别。我们报告两例这种罕见疾病的病例,以说明其临床谱;一例为22岁女性,累及下颌下淋巴结、鼻中隔和声门下区域;另一例为45岁男性,有明显的皮肤受累。

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