A single case of rosai-dorfman disease marked by pathologic fractures, kidney failure, and liver cirrhosis treated with single-agent cladribine.
Rosai-Dorfman disease (RDD) is a proliferative histiocytic disorder of unknown etiology, which is characterized by sinus histiocytosis with massive lymphadenopathy (1). 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.
Department of Leukemia, The University of Texas MD Anderson Cancer Center , Houston, TX , USA.
Koji
K
Sasaki
Department of Leukemia, The University of Texas MD Anderson Cancer Center , Houston, TX , USA.
Naveen
N
Pemmaraju
Department of Lymphoma and Myeloma, The University of Texas MD Anderson Cancer Center , Houston, TX , USA.
Jason R
JR
Westin
Department of Pathology, The University of Texas MD Anderson Cancer Center , Houston, TX , USA.
Wei-Lien
WL
Wang
Department of Hematopathology, The University of Texas MD Anderson Cancer Center , Houston, TX , USA.
Joseph D
JD
Khoury
Department of Nuclear Medicine, The University of Texas MD Anderson Cancer Center , Houston, TX , USA.
Donald A
DA
Podoloff
Department of Orthopedic Oncology, The University of Texas MD Anderson Cancer Center , Houston, TX , USA.
Bryan
B
Moon
Department of Leukemia, The University of Texas MD Anderson Cancer Center , Houston, TX , USA.
Naval
N
Daver
Department of Leukemia, The University of Texas MD Anderson Cancer Center , Houston, TX , USA.
Gautam
G
Borthakur
CD163; Rosai–Dorfman disease; S-100; cladribine; histiocytes