| Literature DB >> 7378987 |
D R Ambruso, T Hays, W J Zwartjes, D G Tubergen, B E Favara.
Abstract
Lymphohistiocytic reticulosis with phagocytosis is a rare, familial disorder affecting infants and children. It is characterized by fever, pancytopenia, hepatosplenomegaly, and a rapidly fatal course. Prior attempts to treat this disease have been unsuccessful. We describe two patients with lymphohistiocytic reticulosis with phagocytosis and hyperlipidemia. A sibling of one patient had died of the same disease. One patient also had abnormal lymphocyte response to mitogens. Both patients who were treated with epipodophyllotoxin VP 16-213 (VP-16) had remission of their disease and resolution of hyperlipidemia. VP-16 appears to be an effective agent for treating lymphohistiocytic reticulosis with phagocytosis.Entities:
Mesh：
Substances：
Year: 1980 PMID： 7378987 DOI： 10.1002/1097-0142(19800515)45:10<2516::aid-cncr2820451008>3.0.co;2-v
Source DB: PubMed Journal: Cancer ISSN： 0008-543X Impact factor: 6.860