Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Nine patients with an acute disease characterized by high fever, loss of weight, prominent hepatosplenomegaly, slight or no lymphadenopathy, abnormal liver function tests, and profound pancytopenia are reported. In all cases, the disease presented in the absence of any pre-existing disease or immunosuppressive therapy. In seven of the nine patients, survival was very short (mean = 7 weeks). Two patients are still alive: one had a relapse 24 months after the initial diagnosis, while the other is in complete remission. The main pathological feature was the infiltration of the marrow, spleen and liver by neoplastic T cells, accompanied by an exuberant hyperplasia of benign-looking, hemophagocytizing histiocytes. The term "peripheral T-cell lymphoma with hemophagocytic syndrome" is proposed for this condition. Retrospective analysis of stored paraffin material (1949 to 1965) from the Radcliffe Infirmary files suggests that at least some of the cases designated as "histiocytic medullary reticulosis" by Scott and Robb-Smith were examples of the syndrome herein described.
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PMID:Peripheral T-cell lymphoma associated with hemophagocytic syndrome. 224 37

The acute phase of Chagas disease lasts 4-8 weeks and is characterized by microscopically detectable parasitaemia. Symptoms are usually mild with severe acute disease occurring in less than 1% of patients. Orally transmitted Trypanosoma cruzi outbreaks can have more severe acute morbidity and higher mortality than vector-borne infection. Congenital T. cruzi infection occurs in 1-10% of infants of infected mothers. Most congenital infections are asymptomatic or cause non-specific signs, requiring laboratory screening for detection. A small proportion of congenital infections cause severe morbidity with hepatosplenomegaly, anaemia, meningoencephalitis and/or respiratory insufficiency, with an associated high mortality. Infected infants are presumed to carry the same 20-30% lifetime risk of cardiac or gastrointestinal disease as other infected individuals. Most control programs in Latin America employ prenatal serological screening followed by microscopic examination of cord blood from infants of seropositive mothers. Recent data confirm that polymerase chain reaction (PCR) is more sensitive and detects congenital infections earlier than conventional techniques. For infants not diagnosed at birth, conventional serology is recommended at at 6 to 9 months of age. In programs that have been evaluated, less than 20% of at risk infants completed all steps of the screening algorithm. A sensitive, specific and practical screening test for newborns is needed to enable Chagas disease to be added to newborn screening programs.
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PMID:Acute and congenital Chagas disease. 2182 May 50