Hemophagocytic lymphohistiocytosis (HLH) is a hyper-inflammatory clinical syndrome associated with neoplastic disorders especially lymphoma, autoimmune conditions, and infectious agents including bacteria, viruses, protozoa and fungi. In both human and veterinary medicine, hemophagocytic histiocytic disorders are clinically important and frequently fatal. HLH in humans can be a primary (familia...

OBJECTIVES To compare the pro-inflammatory cytokine profiles and the cytokine kinetics in patients with secondary macrophage activation syndrome (MAS) due to systemic-onset juvenile idiopathic arthritis (s-JIA) and in both active and inactive disease states of s-JIA (but no MAS), with those demonstrated in EBV-induced haemophagocytic lymphohistiocytosis (HLH) and Kawasaki disease (KD), and to i...

Hemophagocytic Lymphohistiocytosis (HLH) in coronavirus disease 2019 (COVID-19) is a recognized complication of severe illness. However, this phenomenon has been reported most often the setting acute infection. Here we present case patient with history respiratory syndrome Coronavirus 2 (SARS-CoV-2) infection that subsequently developed HLH weeks after treatment COVID-19 and discharges from hos...

Hemophagocytic lymphohistiocytosis (HLH) is an unusual syndrome characterized by fever, splenomegaly, jaundice, and the pathologic finding of hemophagocytosis (phagocytosis by macrophages of erythrocytes, leukocytes, platelets, and their precursors) in bone marrow and other tissues. HLH may be diagnosed in association with malignant, genetic, or autoimmune diseases but is also prominently linke...

Hemophagocytic lymphohistiocytosis (HLH), while uncommon, may be a devastating complication of lymphoma and/or human immunodeficiency virus (HIV) infection. While several of the diagnostic criteria for HLH are relatively nonspecific, particularly in the setting of a systemic inflammatory response, more diagnostic specificity may be achieved with marked elevations in serum ferritin (e.g., >100,0...

The association between nephrotic syndrome (NS), hemophagocytic lymphohistiocytosis (HLH), and certain paraneoplastic syndromes has been documented in the literature. However, nephrotic changes as part of paraneoplastic syndromes are rare in lymphoid malignancies, particularly in non-Hodgkin lymphoma. We report the sudden onset of acute renal failure and NS in a 14-year-old male who initially p...

Hemophagocytic lymphohistiocytosis (HLH) is a disorder of immune dysregulation secondary to a massive unregulated cytokine storm and its downstream consequences. HLH is being increasingly recognized as a cause of pyrexia of unknown origin, unexplained cytopenias, and hepatic dysfunction. However, this potentially treatable condition is often missed due to lack of suspicion, variable, and nonspe...

Hemophagocytic lymphohistiocytosis (HLH) is a rare histiocytic disorder characterized by reactive hyperplasia of the mononuclear phagocytic system, which primarily caused dysfunction cytotoxic killer cells and natural cells, leading to antigen clearance barriers overactivation system due continuous stimulation. HLH encompasses group clinical syndromes marked overproduction inflammatory cytokine...

SIR, I read with interest the case report of a 1-yr-old girl with systemic juvenile idiopathic arthritis and Kikuchi’s disease, who later developed haemophagocytic lymphohistiocytosis (HLH), and its successful management [1]. Diagnostic criteria for HLH were published in 1991 [2]. The diagnosis of HLH requires the presence of all five criteria (fever >38.5 C for 7 or more days, palpable splenom...

Inherited defects of granule-dependent cytotoxicity led to the life-threatening immune disorder hemophagocytic lymphohistiocytosis (HLH), characterized by uncontrolled CD8 T-cell and macrophage activation. In a cohort of HLH patients with genetic abnormalities expected to result in the complete absence of perforin, Rab27a, or syntaxin-11, we found that disease severity as determined by age at H...