Congenital Rosai-Dorfman disease without lymphadenopathy.
Rosai-Dorfman disease (RDD) is a rare histiocytic proliferative disorder with massive lymphadenopathy. We here describe RDD of a neonate who presented with paleness and hepatosplenomegaly but not lymph-node swelling. Routine laboratory studies showed anemia, thrombocytopenia, and an elevated value of gamma-glutamyl transpeptidase. Histological examination of the liver revealed a proliferation of histiocytes with abundant eosinophilic cytoplasm, which were positive for S-100 protein and CD68 but not CD1a and did not reveal Birbeck granules. Radiological studies showed hepatosplenomegaly and a narrowing of the hepatic vein, which might have contributed to hypersplenism resulting in anemia and thrombocytopenia. This case is thought to be congenital RDD without lymphadenopathy.
Iwabuchi
Haruko
H
Division of Pediatrics, Department of Homeostatic Regulation and Development, Niigata University Graduate School of Medical and Dental Sciences, Asahimachi-dori 1, Niigata, 951-8510, Japan.
Kakihara
Toshio
T
Tanaka
Atsushi
A
Uchiyama
Makoto
M
Shibuya
Hiroyuki
H
Umezu
Hajime
H
D003937:Diagnosis, Differential; D006644:Histiocytes / Q000166:cytology / Q000378:metabolism; D015618:Histiocytosis, Sinus / Q000151:congenital* / Q000175:diagnosis / Q000473:pathology / Q000503:physiopathology*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008099:Liver / Q000166:cytology / Q000473:pathology / Q000503:physiopathology; D008206:Lymphatic Diseases / Q000473:pathology / Q000503:physiopathology*; D008264:Macrophages / Q000166:cytology / Q000378:metabolism; D008297:Male