Galicier Lionel, Boutboul David, Oksenhendler Éric, Fieschi Claire, Meignin Véronique
Sorbonne Paris Cité, université Paris Diderot, hôpital Saint-Louis, service d'immunopathologie clinique, 1, avenue Claude-Vellefaux, 75010 Paris, France.
Sorbonne Paris Cité, université Paris Diderot, hôpital Saint-Louis, service d'immunopathologie clinique, 1, avenue Claude-Vellefaux, 75010 Paris, France.
Presse Med. 2017 Jan;46(1):107-116. doi: 10.1016/j.lpm.2016.01.015. Epub 2016 Feb 26.
Rosai-Dorfman sinusal histiocytosis is a benign histiocytic disorder presenting as large lymphadenopathies, mostly in the cervical area. Visceral involvement is not unusual. The diagnosis is based on histologic findings, showing intrasinusoidal histiocytic infiltration with pathognomonic emperipolesis features. Histiocytes display a normal activated phenotype. It probably represents a heterogeneous group of diseases with some common histopathologic findings. An association with immunologic abnormalities or auto-immune diseases, mostly auto-immunes cytopenias is possible. This is a criterion of poor prognosis. The evolution can sometimes be spontaneously favorable. Nonetheless, there is a risk of compression, due to the huge volume of tumoral masses, especially in case of orbital or epidural involvement. Therapeutic abstention is usually justified. Treatments are discussed in threatening forms, evolving forms or in case of poor prognosis factors. Treatment, when indicated, is not codified. Surgery, corticosteroids, immunosuppressive agents and/or alpha interferon can be used.
罗萨伊-多夫曼鼻窦组织细胞增生症是一种良性组织细胞疾病，表现为大多位于颈部的巨大淋巴结病。内脏受累并不罕见。诊断基于组织学发现，显示窦内组织细胞浸润并具有特征性的血细胞吞噬现象。组织细胞表现出正常的活化表型。它可能代表一组具有一些共同组织病理学发现的异质性疾病。与免疫异常或自身免疫性疾病相关，主要是自身免疫性血细胞减少症是有可能的。这是预后不良的一个标准。病情有时可能会自发好转。然而，由于肿瘤块体积巨大，存在压迫风险，尤其是在眼眶或硬膜外受累的情况下。通常不主张进行治疗。在病情呈威胁性、进展性或存在预后不良因素的情况下会讨论治疗方法。如有指征，治疗方法尚无统一规范。可采用手术、皮质类固醇、免疫抑制剂和/或α干扰素。
