DOI: 10.12890/2017_000605
Sinus Histiocytosis with Massive Lymphadenopathy (Rosai Dorfman Disease) and Anaplastic Large Cell Lymphoma
Authors
kapilgarg81@gmail.com
Keywords
Sinus histiocytosis with massive lymphadenopathy, Rosai Dorfman disease, anaplastic large cell lymphoma, immunohistochemistryAbstract
Sinus histiocytosis with massive lymphadenopathy (SHML) is a rare and benign source of lymphadenopathy first described in 1969. This disease commonly presents in children and young adults with supra-diaphragmatic lymphadenopathy mainly at cervical nodal sites, composed of a polyclonal population of histiocytes. Since its description greater than 400 cases have been described, however the literature is quiet scanty when it comes to its association with lymphomas. This case report describes a case with co-existing SHML and anaplastic large cell lymphoma (ALCL). The diagnosis of SHML in our patient did not alter the clinical outcome and patient responded well to treatment of ALCL. Clinicians should maintain a high index of suspicion in cases of infra-diaphragmatic SHML for the presence of occult lymphoma.
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References
- Shi Y, Griffin AC, Zhang PJ, Palmer JN, Gupta P. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): a case report and review of 49 cases with fine needle aspiration cytology. Cytojournal2011;8:3.
- Tilly H, Gaulard P, Lepage E, Dumontet C, Diebold J, Plantier I, et al. Primary anaplastic large-cell lymphoma in adults: clinical presentation, immunophenotype, and outcome. Blood 1997;90:3727–3734.
- Garel L, Lucaya J, Piqueras J. Clinical quiz. Large-cell anaplastic lymphoma coexisting with Rosai-Dorfman disease. Pediatr Radiol 2004;34:509–510.
- Lu D, Estalilla OC, Manning JT, Medeiros LJ. Sinus histiocytosis with massive lymphadenopathy and malignant lymphoma involving the same lymph node: a report of four cases and review of the literature. Mod Pathol 2000;13:414–419.
- Moore JC, Zhao X, Nelson EL. Concomitant sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) and diffuse large B-cell lymphoma: a case report. J Med Case Rep 2008;2:70.