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)

High prevalence rates of hepatitis C virus (HCV) were recently reported among Egyptian blood donors. To confirm these observations and estimate the magnitude of HCV infection in this country, we determined the prevalence of antibodies to HCV (anti-HCV) in samples collected in 1992 from seven different populations of children and adults living in Egypt. Anti-HCV was found in 12.1% of rural primary schoolchildren, 18.1% of residents of a rural village, 22.1% of army recruits, 16.4% of children with hepatosplenomegaly, 54.9% of hospitalized, multitransfused children, 46.2% of adults on hemodialysis, and 47.2% of adults with chronic liver disease or hepatoma. Age-related prevalence of anti-HCV in a random sample of 270 inhabitants of a rural village increased progressively from zero in those 5-10 years of age to 41% in adults greater than the age of 50. Although the increased prevalence of anti-HCV among children and adults with parenteral exposures and chronic liver disease was expected, the prevalence of anti-HCV among persons representing the general population of Egypt was strikingly high. These data demonstrate the magnitude of HCV infection and its importance in chronic liver disease in Egypt. Future studies are needed to determine the routes of transmission of HCV in this country.
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PMID:High seroprevalence of hepatitis C infection among risk groups in Egypt. 752 86

A 23-yr-old male with acute hepatitis A and coexistent hepatitis C became critically ill 3 wk after the onset of fever and jaundice with progressive anemia, thrombocytopenia, and hepatosplenomegaly. Bone marrow biopsy revealed hemophagocytosis. Despite aggressive supportive treatment with parenteral steroids, the patient died of disseminated intravascular coagulopathy with gastrointestinal bleeding. Necropsy of the liver showed histiocyte aggregation in the portal area with hemophagocytosis.
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PMID:Acute hepatitis A with coexistent hepatitis C virus infection presenting as a virus-associated hemophagocytic syndrome: a case report. 967 68

Although seen rarely in Switzerland, schistosomiasis is a parasitosis affecting 200 to 250 million people round the world, mainly in tropical and subtropical regions of Africa, Asia, Central and South America. Depending on the parasitic species, the ureters and the bladder (S. haematobium) or the intestine and the liver (S. mansoni, S. japonicum, S. mekongi) are primarily involved. Other organs may be affected (lung, kidneys and central nervous system). Hepatosplenic schistosomiasis represents a special form of chronic infection by S. mansoni, S. japonicum or S. mekongi predominantly occurring in adolescents heavily and repeatedly infected during childhood, together with an additional genetic predisposition for the disease. Hepatosplenic schistosomiasis on a worldwide scale is one of the most prevalent causes of portal hypertension in man. We describe a 33-year-old Portuguese female with mansonian hepatosplenic schistosomiasis 12 years after leaving Africa, who had hepatosplenomegaly, portal hypertension, esophageal varices and hypersplenism. Splenomegaly and slight anemia had been known for years without prompting further work-up. Two months before diagnosis she had been delivered of a normal child after pregnancy without portal-hypertensive complications, namely esophageal hemorrhage. Because of placenta accreta, however, erythrocyte transfusion had been performed after delivery and was possibly responsible for hepatitis C found later on. Pathophysiology, clinical findings and therapy of the disease are discussed.
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PMID:[Hepatosplenic schistosomiasis: case report and clinical review]. 825 82

We report a 75-year-old Japanese man infected with hepatitis C virus (HCV) who died of acute hepatic failure due to the hepatic infiltration of B-cell non-Hodgkin's lymphoma (NHL) cells. He suddenly developed jaundice, fatigue, fever, and hepatosplenomegaly during the course of chronic infection with HCV. Postmortem liver necropsy revealed extensive infiltration of lymphoma cells into the liver. Although the association between HCV infection and NHL has recently become a matter of concern, we believe this to be the first reported case of acute hepatic failure caused by hepatic involvement of non-Hodgkin's lymphoma in an HCV-infected patient.
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PMID:Acute hepatic failure due to hepatosplenic B-cell non-Hodgkin's lymphoma in a patient infected with hepatitis C virus. 985 65

Cell mediated immune response (CMIR) was studies in 120 patients having chronic liver diseases. Patients were divided into 6 groups, (20 each). (1) Early hepatosplenic Schistosomiasis. (EHSS), (2) Late hepatosplenic Schistosomiasis. (LHSS), (3) Hepatosplenic Schistosomiasis with hepatitis B and/or C infections, (4) Hepatitis B virus cases. (HBV), (5) Hepatitis C virus cases (HCV), (6) Hepatocellular carcinoma cases. (HCC). Twenty within normal subjects taken as controls. Laboratory investigations revealed significant esinophilia in patients of group (1), haemoglobin level was significantly reduced in patients of group (1, 2, 3, & 6), serum albumin was significantly reduced in group (2). The percentage of positivity of skin testing using purified protein derivative, ranged between 10% of patients with LHSS, HBV, HCC and HSS with HBV and/or HCV, 20% of patients with HCV and 25% of patients with EHSS. Percentage of positivity in control group was 100%. The mean diameter of delayed intradermal reaction (2.2 +/- 0.5-6.1 +/- 2.1 mms.) was significantly lower in patients than controls. The response of lymphocyte transformation test to phytohaemmagglutinin was significantly lower in patients when compared to controls. The association of HBV and/or HCV with hepatosplenomegaly was accompanied with a marked depression in cell mediated immune response. Anaemia, hypoalbuminemia and nutritional status of the patients with chronic liver diseases play a major role in the suppression of cell mediated immune response.
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PMID:Cell mediated immune response in chronic liver diseases: schistosomal, viral and neoplastic. 991 13

A 75-year-old man developed subacute progressive muscle weakness and painful paresthesia of the left upper and right lower limbs. The patient had no history of diabetes mellitus. On physical examination, there was no evidence of icterus or hepatosplenomegaly. Palmar erythema without rash was noted. Neurologic examination revealed muscle atrophy and weakness in the left upper limb and mild muscle weakness in the right proximal lower limb. Dysesthesia, severe hypesthesia, and hypalgesia were found in the left upper limb. The tendon reflexes were decreased in the left upper limb and absent in the lower limbs. The cranial nerves were preserved on the day of admission, followed by the involvement of the right oculomotor nerve. Serological examination revealed a mixed IgG/IgM cryoglobulinemia and hepatitis C virus (HCV) infection with evidence of HCV virus replication by PCR for HCV RNA. The patient was diagnosed as having a mixed cryoglobulinemic neuropathy associated with HCV infection. Interferon-alpha therapy with 3 million units subcutaneously was initiated three times per week; however, there was no clinical improvement, although cryoglobulins became undetectable and the level of serum HCV RNA decreased remarkably. Intravenous immunoglobulin therapy 20 g per day for 5 days was also ineffective. The patient developed right facial nerve palsy, followed by right abducens nerve palsy. Treatment with prednisolone 40 mg per day improved and stabilized neurologic symptoms. Although interferon-alpha is considered to be a promising therapy for neurologic complications of HCV infection with mixed cryoglobulinemia, the optimal treatment remains unestablished.
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PMID:[Peripheral neuropathy involving cranial nerves in a patient with hepatitis C virus infection and mixed cryoglobulinemia]. 1108 99

Acquired factor X deficiency has been described in patients with amyloidosis but acquired factor V deficiency is quite rare. We report here a case of life-threatening bleeding and acquired factor V deficiency associated with primary amyloidosis. A 50-year-old man who had no previous hemorrhagic diathesis was referred to our hospital because of recurrent epistaxis, gingival bleeding and hemospermia. The laboratory examination revealed that both the prothrombin time (PT) and the activated partial thromboplastin time (aPTT) were significantly prolonged, and factor V activities were markedly decreased to 14-39% of the normal value. Other coagulation factors such as fibrinogen, prothrombin, factor VII, factor VIII, factor IX and factor X were subnormal and normal. Transaminases were slightly elevated but serological tests of hepatitis B and hepatitis C were negative. Mild hepatosplenomegaly was noted without sign of liver cirrhosis. The PT and aPTT obtained 8 years ago when he received a cholecystectomy due to cholecystitis were both normal. Specific assays for the detection of factor V inhibitor were repeatedly performed but no factor V inhibitor was found. Furthermore, a significant recovery of the infused factor V was noted shortly after an intravenous administration of 5-10 U fresh frozen plasma, but it did not last more than 6 h. Melena, bleedings into the left shoulder and buttock, and finally mortal retroperitoneal hemorrhage developed despite repeated infusions of large amounts of fresh frozen plasma. Acquired factor V deficiency associated with primary amyloidosis was suspected but histological diagnosis was not obtained because of the severe bleeding tendency. Autopsy revealed hepatosplenomegaly and massive deposits of AL amyloid in the liver, spleen, heart and other parenchymal organs. Perivascular amyloid deposition and factor V deficiency are both thought to be the cause of the severe hemorrhagic tendency seen in this patient.
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PMID:Life-threatening bleeding and acquired factor V deficiency associated with primary systemic amyloidosis. 1219 8

Chronic eosinophilic leukemia is a rare entity, characterized by eosinophilia of >1.5 x 10(9)/L, persisting for >6 months after exclusion of other reactive and neoplastic causes of eosinophilia, and occurring in association with a clonal cytogenetic abnormality. Various chromosomal abnormalities have been associated with chronic eosinophilic leukemia. Partial deletion of the long arm of chromosome 16 is a cytogenetic abnormality first reported 20 years ago in patients with acute myeloid leukemia associated with bone marrow eosinophilia (AML-M4Eo). We report a case of a 45-year-old African-American male with hepatitis C and sustained peripheral blood eosinophilia who presented with gross hematuria, dyspnea on exertion, chills, decreased appetite and weight loss of 40 pounds associated with hepatosplenomegaly and lymphadenopathy. Bone marrow biopsy showed clonal cytogenetic abnormality consisting of deletion of the long arm of chromosome 16 (16q22). Philadelphia chromosome t (9;22) and polymerase chain reaction (PCR) analysis for C-kit and platelet-derived growth factor receptor-alpha (PDGFRA) mutations were negative. The patient was treated with imatinib at 400 mg/d with improvement of symptoms, reduction of lymphadenopathy and normalization of the eosinophil count. To our knowledge, this is the first case report of isolated del (16) (q22), a cytogenetic abnormality associated with AML-M4Eo, occurring in chronic eosinophilic leukemia. Whether this cytogenetic abnormality might represent a prodromal phase of AML-M4Eo is not known. In addition, the role of hepatitis C in inducing clonal proliferation of eosinophils is unclear.
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PMID:Case of chronic eosinophilic leukemia with deletion of chromosome 16 and hepatitis C. 1691 38

Cryoglobulinaemia is a systemic inflammatory condition characterised by immune complex-mediated small-to-medium-sized vasculitis. It has a wide variety of presentations ranging from bruising, neuropathy, and hepatosplenomegaly to acute renal failure. Mixed cryoglobulinaemia is the most common type and is strongly associated with hepatitis C. Management approaches include use of cyclophosphamide, prednisolone, and plasmapheresis, with differing views on alternative treatments in resistant cases. Rituximab has emerged as an attractive option in resistant cases on account of its potent immunosuppressive effects on B cells. We describe a case of type 2 mixed cryoglobulinaemia in association with non-Hodgkin's lymphoma resistant to standard treatments which responded well to rituximab. This case is remarkable as mixed cryoglobulinaemia associated with non-Hodgkin's lymphoma presenting with nephritis is unusual, and, contrary to the high rate of recurrence in lymphoma-related cryoglobulinaemia, our patient has not shown any recurrence over 24 months. This highlights an alternative treatment modality which can be used in patients not responsive to existing managements for this condition with benefits of minimal side effects and no oncogenetic potential.
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PMID:Successful treatment of cryoglobulinaemia with rituximab. 2319 59

Haemophagocytic syndrome (HS) is a rare disease that is often fatal despite treatment. HS is characterized by fevers, lymphadenopathy, hepatosplenomegaly, cytopenias and hyperferritinaemia due to deregulated activation and proliferation of macrophages, leading to uncontrolled phagocytosis of platelets, erythrocytes, lymphocytes, and their hematopoietic precursors throughout the reticuloendothelial system. Mycobacterium tuberculosis-associated HS is a rare and underdiagnosed association with only 39 cases reported. We describe a case of HS associated with disseminated Mycobacterium tuberculosis in the setting of post-liver transplantation anti-hepatitis C therapy with pegylated interferon (pegIFN), ribavirin (RBV) and telaprevir (TVR). Despite the delay in the etiologic diagnosis, the patient was treated properly with corticosteroids, cyclosporine and tuberculostatic agents. It is unknown whether telaprevir, a drug that only recently has been started off-label in liver transplant recipients, may have contributed to the development of the HS. Unfortunately, as in many reported cases of HS, the outcome was unfavourable resulting in the death of the patient.
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PMID:Haemophagocytic syndrome in a liver transplant patient during treatment with Telaprevir. 2411 30


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