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

The authors report three patients diagnosed with EBV associated HPCS. The first case died of a fatal EBV infection. The second and third cases had primary disease of malignant lymphoma. In case 2, T cell lymphoma associated HPCS was diagnosed early. However, despite the aggressive treatment of HPCS and T-cell lymphoma, the patient died because of the refractory lymphoma. In case 3, HPCS and B malignant lymphoma were diagnosed at post mortem. EBV was found very late in all three cases. Case 1 and case 2 had a very high DNA-EBV load in blood. Case 3 demonstrated EBV-RNA encoded antigen (EBER) in lymph node by in situ hybridization technique. The clinical features of HPCS were analysed. Four early manifestations in these three cases were emphasized, namely fever, splenomegaly, progressive pancytopenia and impaired liver function test without severe jaundice. The authors stress the most important factor to save the life of the patients is to give early diagnosis and early proper management of HPCS as well as the etiologic diseases. The treatment of choice of early HPCS are pulse corticosteroid, IVIgG. Combination immunochemotherapy including pulse corsticosteroid, IVIgG, cyclosporin A, etoposide and plasma exchange should be given promptly in severe cases. From the present report, it indicates that the association of EBV with HPCS is not uncommon in Thailand. EBV is very important because it gives a very poor prognosis either by being an etiologic cause of HPCS or by association with ML with HPCS. Clinicians should be aware of EBV and recognize it early. The early treatment of EBV should helpfully changes the prognosis of the patients. The role of EBV on the occurrence of HPCS and T-ML is also discussed
 ...
PMID:EBV and hemophagocytic syndrome: analysis of 3 cases, with speculation on clinical features, therapy and role of EBV. 1547 5

Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) is a non-malignant proliferative disorder characterized by histiocytic proliferation and hemophagocytosis following Epstein-Barr virus infection. Though quite rare, this condition represents an often fatal disease primarily affecting the pediatric population. We discuss the case of an adolescent female who presented initially with persistent cervical lymphadenopathy and the typical findings of tonsillar hypertrophy, pharyngitis, and splenomegaly associated with infectious mononucleosis. This case study outlines the pathogenesis, common clinical findings, diagnostic criteria, and a review of the HLH-94 treatment protocol. Early recognition and treatment is emphasized because of the fulminant course of the disorder.
 ...
PMID:Persistent cervical lymphadenopathy in an adolescent with Epstein-Barr induced hemophagocytic syndrome: manifestations of a rare but often fatal disease. 1591 Oct 25

A 28-year-old male was admitted to our hospital with tonsillitis and jaundice. Laboratory findings showed leukocytosis (rate of atypical lymphocytes was 40%), liver dysfunction and hyperbilirubinemia. Epstein-Barr virus (EBV) viral capsid antigen (VCA) IgM and IgG antibodies were positive, and EB nuclear antigen (EBNA) antibody was negative. Abdominal ultrasonography demonstrated hepato-splenomegaly and swelling of intraperitoneal lymph nodes. A diagnosis of infectious mononucleosis was made due to EBV infection. Conservative therapy was given. Total bilirubin and alkaline phosphatase increased to maximum levels of 10.2 mg/dl and 1,590U/l. A liver biopsy specimen revealed infiltration of lymphocytes in sinusoids and portal areas, focal necrosis and intrahepatic cholestasis in parenchyma. Liver function tests returned to normal limits and EBV VCA IgM antibody became negative within 10 weeks from onset.
 ...
PMID:[A case of infectious mononucleosis complicated with severe jaundice]. 1597 53

Most individuals acquire Epstein-Barr virus (EBV) infection in young age. Because of uncommon presentation and misdiagnosis, clinical manifestations are less well described in older age. We present two cases of elderly patients with predominant symptoms attributed to cold agglutinin haemolytic anaemia due to acute EBV infection without fever, lymphadenopathy, pharyngitis or splenomegaly. We conclude that misleading clinical manifestations are frequent in older individuals and may lead to inappropriate diagnostic invasive procedures.
 ...
PMID:Acute Epstein-Barr virus infection in two elderly individuals. 1640 35

Epstein-Barr virus (EBV) is a herpesvirus whose only reservoir host is the human. It is transmitted by oropharyngeal secretions. Primary EBV infection is usually asymptomatic, but sometimes it causes infectious mononucleosis with fever, lymphadenopathies, splenomegaly and pharyngitis. Acute infection is diagnosed by serology (heterophile or specific antibodies). Immunofluorescence and molecular biologic techniques may be used to demonstrate the presence of EBV in biopsy specimens. Mild and transient elevations of serum aminotransferases are common, thus liver biopsy is usually not necessary to confirm the diagnosis. Severe cholestasis is rare (5%). We describe a patient with cholestatic hepatitis and acute EBV infection with atypical lymphocytes and positive anti-VCA IgM. The patient had taken drugs (ibuprofen, paracetamol and valerian). The bad evolution of the patient, the history of exposure to drugs, and the few cases of cholestatic hepatitis due to EBV infection reported, led us to consider liver biopsy. Molecular biologic techniques confirmed the presence of EBV in liver tissue however histologic features did not exclude the toxic aetiology or the concomitant effect of drugs and EBV infection.
 ...
PMID:[Epstein-Barr virus infection and acute cholestatic hepatitis]. 1713 11

Most adults have persistent Epstein-Barr virus (EBV)-infection. Adolescents and young adults with primary EBV-infection frequently develop infectious mononucleosis. Latent EBV-infection is associated with various diseases, neoplasms, and hematological disorders. In vivo animal models of human EBV infection, such as non-human primates, have had limited success. A new rabbit model for primary human EBV-infection is described in this study. Seven male rabbits inoculated intravenously with EBV were sequentially imaged by ultrasonography and computed tomography, and examined for anti-EBV-VCA titer and EBV-DNA levels in blood. Six rabbits demonstrated transient splenomegaly, increased anti-EBV-VCA titers and/or EBV-DNA in blood. Transient infiltration of some EBER1-positive lymphocytes was observed in biopsied liver tissues. After splenomegaly, two rabbits tested continuously negative, two alternatively positive and negative, and one consistently positive EBV detection in blood for 470 days. One tested negative for both EBV DNA and splenomegaly. On the 14th day, mild to moderate numbers of EBER1-positive lymphocytes expressing LMP1, EBNA2, or ZEBRA infiltrated mainly in enlarged white pulps of two splenectomized materials. These cells included both B and T cells. EBV clonality analysis revealed an oligoclonal pattern. These indicate that EBV-inoculated rabbits exhibiting heterogenous host reactions are a good model for primary and persistent human EBV infection.
 ...
PMID:A new animal model for primary and persistent Epstein-Barr virus infection: human EBV-infected rabbit characteristics determined using sequential imaging and pathological analysis. 1820 13

Primary Epstein-Barr virus (EBV) infection is often asymptomatic before five years of age, whereas it is more likely to be symptomatic in older children, adolescents and young adults. The classic triad of symptoms including fever, pharyngitis and lymphadenopathy is well known, but the spectrum of clinical manifestations associated with EBV infection is large and continues to expand, including increasing atypical presentations. This article presents the case of a five-year-old female in which only a protracted and severe itch, resistant to antihistamines, caught the attention of the girl's parents, thus allowing the diagnosis of EBV. Furthermore, EBV related splenomegaly and mild hepatitis as well as thrombocytopenia had an atypical prolonged course.
 ...
PMID:Atypical Epstein-Barr-Virus infection in a 5-year-old girl. 1918 8

We previously reported 24 cases of marrow fibrin-ring granuloma (FRG) encountered in 1 institution and concluded that, contrary to previous studies showing marrow FRG as a diagnostic marker for Q fever, Epstein-Barr virus (EBV) was the most common proven cause of such FRG. The present study characterized patients with EBV-associated marrow FRG. We retrospectively reviewed 17 cases of EBV-associated FRG (43% of 40 cases with marrow FRG) diagnosed by bone marrow biopsy. Patients with EBV-associated hemophagocytic lymphohistiocytosis (5/17 patients) and chronic active EBV infection (4/17) constituted 53% of patients with EBV-associated FRG. Three patients had lymphoma without marrow involvement. All patients except 1 presented first with fever; splenomegaly was the next most common clinical finding (12/17). Cytopenia was observed in the 17 cases: anemia in 16, thrombocytopenia in 9, and leukopenia in 8. Patients with EBV-associated FRG showed lower survival outcomes than did patients without EBV (median, 3.0 vs 11.8 months; P = .009). Patients with bone marrow FRG accompanied by fever require careful evaluation to discern whether active EBV infection is involved because the prognosis is poor.
 ...
PMID:Epstein-Barr virus infection associated with bone marrow fibrin-ring granuloma. 2009 40

We report the case of an HIV-positive man who presented with pyrexia of unknown origin. Histological specimens from an inguinal lymph node and liver biopsy gave a diagnosis of Epstein Barr virus (EBV)-positive Hodgkin's lymphoma and haemophagocytic lymphohistiocytosis (HLH), respectively. HLH is a condition characterized by proliferation of activated macrophages that phagocytose leukocytes, erythrocytes and platelets. Clinical features include splenomegaly, fever and pancytopenia, all of which have a wide differential diagnosis in HIV-positive patients. HLH can be caused by infections, malignancy, drugs or autoimmune conditions. There have been a number of reports of HLH in HIV-positive patients, and it can be seen at all stages of HIV infection. HIV, lymphomas, EBV infection and haemophagocytic syndrome have a complicated pathophysiology. Unfortunately, HLH in this setting has a particularly aggressive course, often with a poor outcome. This case highlights the need for awareness of the syndrome to ensure prompt diagnosis and instigation of appropriate treatment.
 ...
PMID:Haemophagocytic lymphohistiocytosis complicating Hodgkin's lymphoma in an HIV-positive individual. 2097 96

X-linked lymphoproliferative syndromes (XLP) are primary immunodeficiencies characterized by a particular vulnerability toward Epstein-Barr virus infection, frequently resulting in hemophagocytic lymphohistiocytosis (HLH). XLP type 1 (XLP-1) is caused by mutations in the gene SH2D1A (also named SAP), whereas mutations in the gene XIAP underlie XLP type 2 (XLP-2). Here, a comparison of the clinical phenotypes associated with XLP-1 and XLP-2 was performed in cohorts of 33 and 30 patients, respectively. HLH (XLP-1, 55%; XLP-2, 76%) and hypogammaglobulinemia (XLP-1, 67%; XLP-2, 33%) occurred in both groups. Epstein-Barr virus infection in XLP-1 and XLP-2 was the common trigger of HLH (XLP-1, 92%; XLP-2, 83%). Survival rates and mean ages at the first HLH episode did not differ for both groups, but HLH was more severe with lethal outcome in XLP-1 (XLP-1, 61%; XLP-2, 23%). Although only XLP-1 patients developed lymphomas (30%), XLP-2 patients (17%) had chronic hemorrhagic colitis as documented by histopathology. Recurrent splenomegaly often associated with cytopenia and fever was preferentially observed in XLP-2 (XLP-1, 7%; XLP-2, 87%) and probably represents minimal forms of HLH as documented by histopathology. This first phenotypic comparison of XLP subtypes should help to improve the diagnosis and the care of patients with XLP conditions.
 ...
PMID:Clinical similarities and differences of patients with X-linked lymphoproliferative syndrome type 1 (XLP-1/SAP deficiency) versus type 2 (XLP-2/XIAP deficiency). 2111 15


<< Previous 1 2 3 4 5 6 7 Next >>