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)

Two children with acute lymphoblastic leukemia presenting with cellulitis and sepsis are described. Both presented as having hemophagocytic syndrome with the manifestations of prolonged fever, jaundice, pancytopenia, coagulopathy and histiocytic proliferation with hemophagocytosis in their bone marrows. It is similar to the virus-associated hemophagocytic syndrome (VAHS), except for the absences of lymphadenopathy, skin rash and hepatosplenomegaly. Concomitant virus infections were excluded in these two cases. Both patients' conditions improved after appropriate antibiotics and intravenous immunoglobulin therapy. The prognosis seemed better in bacteria-associated hemophagocytic syndrome (BAHS) than in VAHS even in immunocompromised patients.
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PMID:Bacteria-associated hemophagocytic syndrome in childhood acute lymphoblastic leukemia: report of two cases. 907 88

A 32-year-old male with chronic hepatitis B was admitted to a hospital with cellulitis in the right leg in September 2006. Pancytopenia, hepatosplenomegaly, and systemic superficial lymph node swelling were noted, and he was referred to our hospital. He developed fever and liver dysfunction in June 2007 and underwent a splenectomy. His pancytopenia subsequently improved. A pathologic diagnosis of hepatosplenic alphabeta T cell lymphoma was made by examining spleen tissue and biopsy specimens of the liver and mesenteric lymph node. He had stage IVB disease because neoplastic T cells were noted in the bone marrow. The response of the lymphoma to conventional chemotherapy including the CHOP (cyclophosphamide, adriamycin, vincristine, prednisolone) and DeVIC (dexamethasone, etoposide, ifoshamide, carboplatin) regimens was poor and transient. A partial remission was obtained with an ESHAP (etoposide, cisplatin, cytarabine, methylprednisolone) regimen. Therefore, we planned a bone marrow transplantation (BMT) from an HLA-haploidentical sibling donor. He was moved to the Department of Hematology, Hyogo Medical College, to receive this BMT as part of a clinical trial. During the conditioning procedure for the transplantation, however, he died of septicemia. Since hepatosplenic alphabeta T cell lymphoma is very rare with only 23 reported cases to date, herein we report this case and discuss the therapeutic strategy.
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PMID:Hepatosplenic alphabeta T cell lymphoma. 2021 52