Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Thirty-two patients with the Felty syndrome, defined by the presence of rheumatoid arthritis, splenomegaly, and neutropenia, have been studied in comparison with 32 patients with rheumatoid arthritis matched for age, sex, and disease duration, and 9 patients with rheumatoid arthritis and idiopathic neutropenia. Patients with the Felty syndrome had severe destructive arthritis, which progressed during follow-up despite little evidence of objective synovitis, and a higher frequency of extra-articular manifestations, including vasculitis. Bacterial infection tended to occur in patients with the lowest neutrophil count but continued to occur in some despite normalization of the WBC. Prognosis was poor and 8 deaths occurred, predominantly from sepsis. Serologic features were prominent. High titers of IgG rheumatoid factor and circulating immune complexes characterized patients with persistent neutropenia. A family history of rheumatoid arthritis was more common in patients with the Felty syndrome. The association with HLA DR4 was very strong; in addition there was an increased frequency of the DQw3 variant, 3b, suggesting that HLA Class II genes in linkage with DR4 may contribute to disease expression.
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PMID:The Felty syndrome: a case-matched study of clinical manifestations and outcome, serologic features, and immunogenetic associations. 196 4

Hyperreactive malarious splenomegaly (HMS), formerly known as tropical splenomegaly syndrome (TSS), was recognized some 20 years ago as an entity distinct from the splenic enlargement resulting directly from malarial parasitaemia. Its basis appears to be a disturbance in the T-lymphocyte control of the humoral response to recurrent malaria, possibly linked to particular HLA Class II antigens. Gross overproduction of IgM antibodies leads to the formation of high molecular weight immune complexes, persistent gross splenomegaly recurrent episodes of profound anaemia and increased susceptibility to infections. Those with gross disease experience a high mortality, which constitutes a major public health problem in communities where the syndrome affects a majority of adults.
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PMID:Hyperreactive malarious splenomegaly (tropical splenomegaly syndrome). 1546 19