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)

Two patients admitted to the hospital because of severe bacterial infection were diagnosed as having visceral leishmaniasis. The types of bacterial infection were perianal abscess and pneumonia; Escherichia coli and Streptococcus pneumoniae were isolated from exudates and blood cultures, respectively. A third patient admitted because of acute necrotizing infection of the pharynx and visceral leishmaniasis is also discussed. Cultures from this patient failed to yield pathogens. Anemia, leukopenia, or thrombocytopenia was present in all patients, and bone marrow aspirate revealed the presence of Leishmania in macrophages. We conclude that in areas where leishmaniasis is endemic, early bone marrow aspirate should, in most instances, be performed in patients with bacterial infection associated with anemia, leukopenia, or thrombocytopenia if hepatomegaly and/or splenomegaly is present.
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PMID:Bacterial infection as a presenting manifestation of visceral leishmaniasis. 219 55

Recurrent macrophage activation syndrome (MAS) is very rare. We present the case of an adolescent boy with human leukocyte antigen (HLA) B27-positive ankylosing spondylitis (AS), who experienced episodes of recurrent MAS since he was a toddler. A 16-year-old boy was admitted because of remittent fever with pancytopenia and splenomegaly after surgical intervention for an intractable perianal abscess. He had been diagnosed with hemophagocytic lymphohistiocytosis (HLH) 4 different times, which was well controlled with intravenous immunoglobulin and steroids since the age of 3. We were unable to identify the cause for the HLH. He remained symptom-free until the development of back pain and right ankle joint pain with swelling at 15 years of age. He was diagnosed with HLA B27-positive AS with bilateral active sacroiliitis. He showed symptom aggravation despite taking naproxen and methotrexate, and the symptoms improved with etanercept. On admission, his laboratory data showed leukopenia with high ferritin and triglyceride levels. Bone marrow biopsy examination showed histiocytic hyperplasia with hemophagocytosis. There was no evidence of infection. He received naproxen alone, and his symptoms and laboratory data improved without any other immunomodulatory medications. Genetic study revealed no primary HLH or inflammasome abnormalities. In this case, underlying autoimmune disease should have been considered as the cause of recurrent MAS in the young patient once primary HLH was excluded.
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PMID:Recurrent macrophage activation syndrome since toddler age in an adolescent boy with HLA B27 positive juvenile ankylosing spondylitis. 2782 29