AUTHOR=Sasaki Koji , Pemmaraju Naveen , Westin Jason R. , Wang Wei-Lien , Khoury Joseph D. , Podoloff Donald A. , Moon Bryan , Daver Naval , Borthakur Gautam 

TITLE=A Single Case of Rosai–Dorfman Disease Marked by Pathologic Fractures, Kidney Failure, and Liver Cirrhosis Treated with Single-Agent Cladribine

JOURNAL=Frontiers in Oncology

VOLUME=4

YEAR=2014

URL=https://www.frontiersin.org/journals/oncology/articles/10.3389/fonc.2014.00297

DOI=10.3389/fonc.2014.00297

ISSN=2234-943X

ABSTRACT=<p>Rosai–Dorfman disease (RDD) is a proliferative histiocytic disorder of unknown etiology, which is characterized by sinus histiocytosis with massive lymphadenopathy (<xref ref-type="bibr" rid="B1">1</xref>). In most cases, RDD has a benign course and treatment is not necessary. However, severe cases of RDD require treatment, and the treatment strategy is determined on the basis of the severity of the disease or the extranodal involvement of vital organs. We report a single case of RDD with atypical presentation of persistent constitutional symptoms, progressing pathologic fractures, and end-organ dysfunction, including acute kidney failure and liver cirrhosis with esophageal varices.</p>