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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Institution: |
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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