Gulwani Hanni, Bhalla Sunita, Srivastava Amit
Department of Histopathology, Sir Ganga Ram Hospital, New Delhi, India.
Indian J Ophthalmol. 2008 Nov-Dec;56(6):507-9. doi: 10.4103/0301-4738.43375.
Rosai-Dorfman-Destombes (sinus histiocytosis with massive lymphadenopathy) disease is an uncommon disease characterized by benign proliferation of histiocytes, with painless lymph node enlargement and frequent extranodal disease. Orbital involvement occurs in 9-11% of cases. However, isolated Rosai-Dorfman-Destombes disease of the lacrimal gland without any systemic involvement is very rare with only three case reports. We describe here one such young male patient with unilateral lacrimal gland swelling. Excision biopsy revealed almost complete replacement of the lacrimal gland by lymphocytes, plasma cells and large pale histiocytes. The latter exhibited emperipolesis and stained positive for S-100 and CD68 on immunohistochemistry. Patient is well and has no other manifestation or recurrence of the disease during a follow-up of 24 months.
罗萨伊-多夫曼-德斯顿贝斯病（伴巨大淋巴结病的窦性组织细胞增生症）是一种罕见疾病，其特征为组织细胞良性增殖，伴有无痛性淋巴结肿大且常伴有结外病变。眼眶受累见于9% - 11%的病例。然而，仅累及泪腺而无任何全身受累的孤立性罗萨伊-多夫曼-德斯顿贝斯病非常罕见，仅有3例病例报告。我们在此描述一名这样的年轻男性患者，其单侧泪腺肿胀。切除活检显示泪腺几乎完全被淋巴细胞、浆细胞和大的淡染组织细胞取代。后者表现为血细胞吞噬现象，免疫组化显示S - 100和CD68呈阳性。患者情况良好，在24个月的随访期间无该疾病的其他表现或复发。
