Juan-Ron fever: a rare case report

Sourya Acharya, Samarth Shukla, Nitin Raisinghani, Rasika Thakare

Abstract


Juan-Ron fever named after Jaun Rosai and Ronald Dorfman is the fever associated with Rosai Dorfman disease also known as Sinus Histiocytosis with Massive Lymphadenopathy (SHML). It is a rare disorder of unknown etiology that is characterized by abundant histiocytes in the lymph nodes throughout the body. Usually patient presents with painless lymphadenopathy. We present a case of a 45 year old male who presented to us with bilateral cervical lymphadenopathy and fever, later on diagnosed to have SHML.


Keywords


Rosai Dorfman disease, SHML, Histiocytes, Lymphadenopathy

Full Text:

PDF

References


Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy. Arch Pathol. 1969;87:63-70.

Juskevicius R, Finlay JL. Rosai-Dorfman disease of the parotid gland, cytologic and histopathologic findings with immunohistochemical correlation. Arch Pathol Lab Med. 2001;125:1348-50.

Foucar E, Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. Sem Diagn Pathol. 1990;7:19-73.

Lauwers GY, Perez-Atayde A, Dorfman RF, Rosai J. The digestive system manifestations of Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy): review of 11 cases. Hum Pathol. 2000;31:380-5.

Ambati S, Chamyan G, Restrepo R, Escalon E, Fort J, Pefkarou A, et al. Rosai-Dorfman disease following bone marrow transplantation for pre-B cell acute lymphoblastic leukemia. Pediatr Blood Cancer. 2008;51:433-5.

Lu D, Estalilla OC, Manning JT Jr, 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. Modern Pathol. 2000;13:414-9.

Tsang WY, Yip TT, Chan JK. The Rosai-Dorfman disease histiocytes are not infected by Epstein-Barr virus. Histopathology. 1994;25:88-90.

McClain LK, Natkunam Y, Swerdlow HS. Atypical cellular disorders. Hematology. 2004;1:283-322.

Foucar E, Rosai J. Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. Semin Diagn Pathol. 1990;7:19-73.

Lauwers GY, Perez-Atayde A, Dorfman RF, Rosai J. The digestive system manifestations of Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy): review of 11 cases. Hum Pathol. 2000;31:380-5.

Antonius JI, Farid SM, Baez-Giangreco A. Steroid-responsive Rosai-Dorfman disease. Pediatr Hematol Oncol. 1996 Nov-Dec;13(6):563-70.

Komp DM. The treatment of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). Semin Diagn Pathol. 1990;7:83-6.