Epstein Barr virus (EBV)-related hemophagocytic lymphohistiocytosis (EBV-HLH) is a form of acquired, infection-related HLH which typically represents a fulminant presentation of an acute EBV infection of CD8+ T cells with 30-50% mortality rate. Systemic EBV-positive lymphoproliferative disease of childhood (SE-LPD) is a rare T cell lymphoproliferative disorder predominantly arising in the setti...

In Caenorhabditis elegans, the dopamine transporter DAT-1 regulates synaptic dopamine (DA) signaling by controlling extracellular DA levels. In dat-1(ok157) animals, DA is not taken back up presynaptically but instead reaches extrasynpatic sites, where it activates the dopamine receptor DOP-3 on choligeneric motor neurons and causes animals to become paralyzed in water. This phenotype is called...

INTRODUCTION Major hyperferritinemia is a rare feature in clinical laboratories associated with a wide variety of disorders, including hemophagocytic lymphohistiocytosis (HLH). The diagnosis of HLH is based on clinical and biological criteria, such as those proposed by the Histiocyte Society. However, several of these criteria are not relevant in the specific setting of hematologic malignancies...

Safeguarding the proteome is central to the health of the cell. In multi-cellular organisms, the composition of the proteome, and by extension, protein-folding requirements, varies between cells. In agreement, chaperone network composition differs between tissues. Here, we ask how chaperone expression is regulated in a cell type-specific manner and whether cellular differentiation affects chape...

OBJECTIVE To study the profile of children with Hemophagocytic lymphohistiocytosis (HLH) and compare the outcome of treatment with intravenous immunoglobulin therapy and Dexamethasone vs. HLH-2004 protocol. METHODS The present retrospective cohort study was conducted in a tertiary care pediatric hospital in Chennai. Children with a diagnosis of HLH admitted to the hospital from June 2008 thro...

Hemophagocytic lymphohistiocytosis (HLH) is a rare cause of cytopenia and is often associated with lymphoma. The occurrence of HLH in the course of lymphoma treatment could be an indicator for refractory disease.

Introduction Macrophage Activation Syndrome (MAS) is a severe complication of rheumatic diseases, frequently associated with systemic juvenile idiopathic arthritis (sJIA), but also described in others pediatric inflammatory disorders including Juvenile Systemic Lupus Erythematosus (SLE) and Kawasaki disease. Due to the close resemblance to a group of histiocytic disorders collectively known as ...

This is a case report with serial imaging showing progression of diffuse osteonecrosis in a patient after a diagnosis of secondary hemophagocytic lymphohistiocytosis (HLH). While bone marrow involvement in HLH has been long noted at histological evaluation and is itself one of the diagnosis criteria, to the best of our knowledge, there has been no previous publication addressing osseous image f...

Hemophagocytic lymphohistiocytosis (HLH) can occur as primary idiopathic syndrome or secondary to neoplastic, infection or autoimmune processes. It is characterized by the proliferation of histiocytes with phagocytosis of formed elements of blood. Clinical manifestations include signs and symptoms of immune activation and pancytopenia. This report presents a child with infection associated with...

Emphysematous pyelonephritis (EPN) and hemophagocytic lymphohistiocytosis (HLH) are rare, fatal illnesses. The presence of both at once in a patient is extremely rare. number reported cases EPN <800 worldwide to date. Contrarily, the prevalence adults with HLH estimated be 1 every 2000 admitted tertiary health center. This case report aims present 45-year-old woman who was diagnosed history ...