title: Successful treatment of lymphohistiocytic reticulosis with phagocytosis with epipodophyllotoxin VP 16-213.
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.
mesh_terms: D001772:Blood Cell Count; D005047:Etoposide / Q000008:administration & dosage*; D005260:Female; D006801:Humans; D006949:Hyperlipidemias / Q000097:blood / Q000188:drug therapy / Q000209:etiology; D007136:Immunoglobulins / Q000032:analysis; D007223:Infant; D008206:Lymphatic Diseases / Q000150:complications / Q000188:drug therapy* / Q000276:immunology; D008213:Lymphocyte Activation; D008297:Male; D010587:Phagocytosis; D011034:Podophyllotoxin / Q000031:analogs & derivatives*
chemical_list: D007136:Immunoglobulins; D005047:Etoposide; D011034:Podophyllotoxin