Ruggiero Antonio, Attinà Giorgio, Maurizi Palma, Mulè Antonino, Tarquini Elisabetta, Barone Giuseppe, Lazzareschi Ilaria, Riccardi Riccardo
Division of Pediatric Oncology, Catholic University, Rome, Italy.
J Pediatr Hematol Oncol. 2006 Feb;28(2):103-6. doi: 10.1097/01.mph.0000200686.33291.d1.
Rosai-Dorfman disease, or sinus histiocytosis with massive lymphadenopathy (SHML), is a rare and self-limiting benign disorder that most commonly involves the cervical lymph nodes. The authors describe two cases of SHML. Fine-needle aspiration of the lymphadenopathy was performed in both patients. Immunocytochemical and histologic features, as the evidence of emperipolesis and S100 protein positivity on immunostaining, were typical of SHML. Fine-needle aspiration cytology plays an important diagnostic role in SHML and may be conclusive in a typical clinical setting. The diagnosis of SHML should be considered in the differential diagnosis of massive, painless cervical lymphadenopathy. Long-term follow-up is necessary to observe the complete regression of the massive lymphadenopathy. However, specific therapy is available and should be limited to patients with compressive symptoms or extranodal disease.
罗萨伊-多夫曼病,即伴巨大淋巴结病的窦性组织细胞增生症(SHML),是一种罕见的自限性良性疾病,最常累及颈部淋巴结。作者描述了两例SHML病例。两名患者均对肿大淋巴结进行了细针穿刺。免疫细胞化学和组织学特征,如免疫染色显示的血细胞吞噬现象和S100蛋白阳性,是SHML的典型表现。细针穿刺细胞学检查在SHML诊断中发挥重要作用,在典型临床情况下可能具有决定性意义。在鉴别诊断巨大无痛性颈部淋巴结病时应考虑SHML的诊断。需要长期随访以观察巨大淋巴结病的完全消退。然而,有可用的特异性治疗方法,且应仅限于有压迫症状或结外病变的患者。