BACKGROUND Epstein Barr virus (EBV) plays a causal role in some diseases, including infectious mononucleosis, lymphoproliferative diseases and nasopharyngeal carcinoma. Detection of EBV infection has been shown to be a useful tool for diagnosing EBV-related diseases. In the present study, we compared the performance of molecular tests, including fluorescence in situ hybridization (FISH) and EBV...

Objectives We evaluated whether the neutralization of IFNg in murin sHLH and fulminant sHLH affected the disease features and we investigated whether treatment with an anti-IFNg mAb affected the clinical and laboratory features in murine models of sHLH and fulminant sHLH; we also exploited the in vivo principle that, in the presence of an anti-IFNg antibody, circulating IFNg bound to the antibo...

PATIENT Male, 30 FINAL DIAGNOSIS: Hemophagocytic lymphohistiocytosis (LHL) Symptoms: Abdominal pain • fever • hypotension • pancytopenia MEDICATION - Clinical Procedure:- Specialty: Infectious Diseases. OBJECTIVE Rare disease. BACKGROUND Hemophagocytic lymphohistiocytosis (HLH) is a result of dysregulated cellular response system. Primary HLH is an autosomal recessive disorder of childhoo...

Chronic active Epstein-Barr virus infection (CAEBV) is a rare disease in which previously healthy persons develop severe, life-threatening illness. Mutations in the perforin gene have been found in familial hemophagocytic lymphohistiocytosis, which shares some features with CAEBV. We studied a patient who died at age 18, 10 years after the onset of CAEBV. The patient had high titers of antibodi...

Haemophagocytic lymphohistiocytosis is a rare but potentially fatal disease. Diagnosing this disease may be difficult and is often delayed because the clinical presentation mimics other conditions like severe sepsis, hepatic failure and malignancies. We reviewed the clinical presentations, response to treatment, and outcomes of children diagnosed with haemophagocytic lymphohistiocytosis from 19...

We present a case of tuberculosis-associated hemophagocytic lymphohistiocytosis in a 14-year-old girl. The patient presented with weight loss, malaise, fatigue, prolonged fever, and generalized lymphadenopathy. Laboratory investigation revealed pancytopenia (white blood cells, 2,020 cells/µL; hemoglobin, 10.2 g/dL; platelets, 52,000 cells/µL), hypertriglyceridemia (229 mg/dL), and hyperferritin...

In four experiments, the accumulation, over time, of a tendency to hear separate high and low streams in a sequence of high (H) and low (L) tones, presented in a galloping rhythm (HLH-HLH-...), was studied. Each trial was composed of two parts, an induction sequence, then a test sequence, with no break between them. The test sequence was always heard at the far left. When the induction sequence...

Hemophagocytic lymphohistiocytosis (HLH) is a rare, fatal disorder of children, affecting predominantly the mononuclear phagocytic system. Previous reports indicate that Epstein-Barr virus (EBV)-associated hemophagocytic lymphohistiocytosis (EBV-HLH) can also be fatal in many cases, although the prognosis for EBV-HLH is better than for the familial form of hemophagocytic lymphohistiocytosis. We...

hemophagocytic lymphohistiocytosis (hlh) is one of the complications of epstein-barr virus (ebv) infection. although the patients who have developed hlh following ebv have normal immune system, there are a few patients with ebv-induced immune deficiency who develop hlh as well. here, we describe the case of a 10-year-old girl with neurological complications caused by ebv-induced hlh. the patien...