Sinus histiocytosis with massive lymphadenopathy Rosai-Dorfman's Disease as cause of isolated hilar lymphadenopathy and complete remission after high dose steroid

Authors

• Hamdan H. Al-Jahdali, Pulmonary Division, Department of Medicine, Mail Code 1443, King Abdulaziz Medical City, King Fahad National Guard Hospital, King Saud University, PO Box 101830, Riyadh 11665, Kingdom of Saudi Arabia. Tel. +966 (1) 2520088 Ext. 14221/14209. Fax. +966 (1) 2520140. E-mail: jahdali{at}yahoo.com
• Nehad N. Al-Shirawi, Pulmonary Division, Department of Medicine, Mail Code 1443, King Abdulaziz Medical City, King Fahad National Guard Hospital, King Saud University, PO Box 101830, Riyadh 11665, Kingdom of Saudi Arabia. Tel. +966 (1) 2520088 Ext. 14221/14209. Fax. +966 (1) 2520140. E-mail: jahdali{at}yahoo.com
• Hana S. Bamefleh, Pulmonary Division, Department of Medicine, Mail Code 1443, King Abdulaziz Medical City, King Fahad National Guard Hospital, King Saud University, PO Box 101830, Riyadh 11665, Kingdom of Saudi Arabia. Tel. +966 (1) 2520088 Ext. 14221/14209. Fax. +966 (1) 2520140. E-mail: jahdali{at}yahoo.com
• Nizar M. Yamani, Pulmonary Division, Department of Medicine, Mail Code 1443, King Abdulaziz Medical City, King Fahad National Guard Hospital, King Saud University, PO Box 101830, Riyadh 11665, Kingdom of Saudi Arabia. Tel. +966 (1) 2520088 Ext. 14221/14209. Fax. +966 (1) 2520140. E-mail: jahdali{at}yahoo.com

Abstract

Rosai-Dorfman9s Disease, also known as sinus histiocytosis with massive lymphadenopathy SHML, is a rare histiocytic proliferative disorder and a distinct clinico-pathological feature of unknown origin. Painless cervical lymphadenopathy is the most common clinical presentation. Different treatment modalities have been tried with variable responses, however, there is no consensus on the best modality of treatment. Here, we present a case report of SHML causing isolated hilar lymphadenopathy with complete remission for more than 6 years, after a short course of high dose steroid dexamethasone 20 mg daily for 3 days.

Article Type

Case Report

First Page

763

Last Page

765

Recommended Citation

Al-Jahdali, Hamdan H.; Al-Shirawi, Nehad N.; Bamefleh, Hana S.; and Yamani, Nizar M. (2008) "Sinus histiocytosis with massive lymphadenopathy Rosai-Dorfman's Disease as cause of isolated hilar lymphadenopathy and complete remission after high dose steroid," Saudi Medical Journal: Vol. 29: Iss. 5, Article 23.
DOI: https://doi.org/10.15537/1658-3175.4339