CONGENITAL ROSAI-DORFMAN DISEASE WITHOUT LYMPHADENOPATHY |
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| Authors: | Haruko Iwabuchi Toshio Kakihara Atsushi Tanaka Makoto Uchiyama Hiroyuki Shibuya Hajime Umezu |
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| Affiliation: | a Division of Pediatrics, Department of Homeostatic Regulation and Development, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.b Department of Clinical Pathology, Niigata City General Hospital, Niigata, Japan.c Department of Surgical Pathology, Niigata University Hospital, Niigata, Japan. |
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| Abstract: | 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 γ-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. |
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| Keywords: | Anemia Liver Neonate Rosai-Dorfman Disease Thrombocytopenia |
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