Hemophagocytic lymphohistiocytosis (HLH) is the result of excessive cytokine release, leading to over-response by immune cells, such as macrophages and T lymphocytes. Here, we report a lethal case of HLH with a complete clinical course. The patient was a 45-year-old man with fever and chills since two months ago plus splenomegaly, hepatomegaly, and pancytopenia. The Anti-HBc IgM was positive, b...

Specific reversed-phase high-performance liquid chromatography conditions are reported for the analysis of recombinant and native human luteinizing hormone (hLH) and human chorionic gonadotropin (hCG) preparations. Heterodimeric hLH, hCG and their alpha- and beta-subunits migrated with significantly different retention times (t(R)) in the following order of increasing hydrophobicity: alpha-hCG<...

Hemophagocytic lymphohistiocytosis (HLH) during childhood cancer treatment is a rare adverse event posing major diagnostic and therapeutic challenges. Between 1995 and 2006, 6 children developed HLH while on conventional chemotherapy (n=4) or after allogeneic stem cell transplantation (n=2). Treatment of HLH included dexamethasone and etoposide, 2 children additionally received infliximab or da...

emophagocytic lymphohistiocytosis (HLH) is a potentially life-threatening disorder characterized by immune dysregulation, overwhelming immune activation, and inflammation. HLH has been categorized as primary or familial HLH (FHLH), when there is a family history of HLH or known underlying genetic defects. Reactive or secondary HLH occurs in the setting of infection or underlying rhematologic di...

Question Macrophage activation syndrome is a severe complication of autoimmune and autoinflammatory disease. MAS is most strongly associated with systemic juvenile idiopathic arthritis (sJIA) but can also be seen in Kawasaki disease, SLE or IBD. Clinically, MAS is strikingly similar to hemophagocytic lymphohistiocytosis (HLH) and the initial differentiation between sJIA-associated MAS and hered...

As an infrequent but potentially life-threatening hyperinflammatory syndrome, hemophagocytic lymphohistiocytosis (HLH) is clinically characterized with prolonged fever, hepatosplenomegaly, cytopenia, hypertriglyceridemia, hyperferritinemia and hemophagocytosis in bone marrow, liver, spleen or lymph nodes. Malignancy-associated HLH (M-HLH), one type of acquired HLH, usually presents variable ove...

BACKGROUND We retrospectively analysed charts of patients with blood ferritin level >5000 µg/l. The aim of the study was to look for the likelihood of haemophagocytic lymphohistiocytosis (HLH) in these patients. METHODS Forty-two patients demonstrated hyperferritinaemia and could be evaluated. The diagnosis of HLH was based on a recently published HScore and an earlier diagnostic algorithm. ...

Background/aim Rheuma-associated hemophagocytic lymphohistiocytosis (Rh-HLH), also called macrophage activation syndrome (MAS), is a severe complication of systemic inflammatory disorders. Rh-HLH has clinical and laboratory similarities to other forms of HLH, and is potentially life threatening. Treatment of Rh-HLH has not been standardized yet, but it commonly includes a variety of agents such...

BACKGROUND Hemophagocytic lymphohistiocytosis (HLH) is a catastrophic syndrome of unrestrained immune activation. Evaluation and management of HLH in the tropics is challenging. OBJECTIVES To examine the reported etiologies and management of HLH reported from the sub-continent. METHODS Systematic review of all published cases from the Indian sub-continent. RESULTS We found only 156 publis...

Abstract Background Haemophagocytic lymphohistiocytosis (HLH) is a rare disorder with poor prognosis characterised by substantial immune activation leading to end‐organ failure. In childhood, genetic defects that impair cytotoxic function of natural killer cells and T cells. are often identified. adults, clinical manifestations similar those observed in children but the aetiology unclear. Aims ...