ROSAI DORFMAN DISEASE WITH EXTENSIVE BONY INVOLVEMENT- A DIAGNOSTIC DILEMMA
Keywords:Pyrexia of unknown origin, Histiocytic disorder, Non Langerhan cell histiocytosis, Immune Mediated
AbstractPreviously classified as Non Langerhan cell histiocytosis by Working Group of Histiocytic Society in 1987 Rosai Dorfman Destombes disease was first described by Destombes in 1965 and later in 1969 by Rosai and Dorfman as a rare histiocytic disorder with sinus histiocytosis and massive lymphadenopathy. They exist as both nodal and extranodal forms. Immunohistochemistry is an essential part of diagnosis as to differentiate between Langerhans cell histiocytosis and another malignant histiocytosis. Some overlap has also been reported with IgG4-related diseases. We hereby reflect upon a patient who presented to our facility with pyrexia of unknown origin, the challenges faced in order to reach a diagnosis and the management offered.
Bruce-Brand C, Schneider JW, Schubert P. Rosai-Dorfman disease: an overview. J Clin Pathol 2020;73(11):697–705.
Foucar E, Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. Semin Diagn Pathol 1990;7(1):19–73.
Parkash O, Yousaf MS, Fareed G. Rosai-Dorfman's disease, an uncommon cause of common clinical presentation. J Pak Med Assoc 2019;69(8):1213–5.
Soomro IN, Hameed K. Arthritis and rosai-dorfman disease of the skin: a diagnostic dilemma. J Pak Med Assoc 1999;49(11):280–2.
Mosheimer BA, Oppl B, Zandieh S, Fillitz M, Keil F, Klaushofer K, et al. Bone involvement in Rosai-Dorfman disease (RDD): a case report and systematic literature review. Curr Rheumatol Rep 2017;19(5):29.
Mallick S, Ghosh R, Iyer VK, Jain D, Mathur SR. Cytomorphological and morphometric analysis of 22 cases of Rosai-Dorfman disease: a large series from a tertiary care centre. Acta Cytol 2013;57(6):625–32.
Demicco EG, Rosenberg AE, Björnsson J, Rybak LD, Unni KK, Nielsen GP. Primary Rosai-Dorfman disease of bone: a clinicopathologic study of 15 cases. Am J Surg Pathol 2010;34(9):1324–33.
Abla O, Jacobsen E, Picarsic J, Krenova Z, Jaffe R, Emile JF, et al. Consensus recommendations for the diagnosis and clinical management of Rosai-Dorfman-Destombes disease. Blood 2018;131(26):2877–90.
Goyal G, Ravindran A, Young JR, Shah MV, Bennani NN, Patnaik MM, et al. Clinicopathological features, treatment approaches, and outcomes in Rosai-Dorfman disease. Haematologica 2020;105(2):348–57.
Copyright (c) 2023 Maria Zahid Ahmed, Madiha Mahmood, Jawaharlal Langhani, Muhammad Zohair, Mahnoor Virk, Muhammad Mubarak, Fazal Akhtar, Ejaz Ahmed
This work is licensed under a Creative Commons Attribution-NoDerivatives 4.0 International License.
Journal of Ayub Medical College, Abbottabad is an OPEN ACCESS JOURNAL which means that all content is FREELY available without charge to all users whether registered with the journal or not. The work published by J Ayub Med Coll Abbottabad is licensed and distributed under the creative commons License CC BY ND Attribution-NoDerivs. Material printed in this journal is OPEN to access, and are FREE for use in academic and research work with proper citation. J Ayub Med Coll Abbottabad accepts only original material for publication with the understanding that except for abstracts, no part of the data has been published or will be submitted for publication elsewhere before appearing in J Ayub Med Coll Abbottabad. The Editorial Board of J Ayub Med Coll Abbottabad makes every effort to ensure the accuracy and authenticity of material printed in J Ayub Med Coll Abbottabad. However, conclusions and statements expressed are views of the authors and do not reflect the opinion/policy of J Ayub Med Coll Abbottabad or the Editorial Board.
USERS are allowed to read, download, copy, distribute, print, search, or link to the full texts of the articles, or use them for any other lawful purpose, without asking prior permission from the publisher or the author. This is in accordance with the BOAI definition of open access.
AUTHORS retain the rights of free downloading/unlimited e-print of full text and sharing/disseminating the article without any restriction, by any means including twitter, scholarly collaboration networks such as ResearchGate, Academia.eu, and social media sites such as Twitter, LinkedIn, Google Scholar and any other professional or academic networking site.