Acquired hemophagocytic lymphohistiocitosis (HLH) syndrome can be a complication of visceral leishmaniasis (VL). A multicenter prospective study was conducted to determine the frequency of HLH syndrome in children with VL. Twenty-four children with VL were identified, and 10 (41%) developed HLH syndrome. VL should be ruled out in all children with HLH criteria living in or coming from endemic a...

Background Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening emergency and any delay in diagnosis and/or treatment is associated with high mortality. It is rarely observed in adult patients. HLH has multifaceted clinical presentations with often non-specific signs and symptoms that are often found in other clinical conditions. Classical manifestations suggestive of HLH include feve...

Hemophagocytic lymphohistiocytosis (HLH) is an unusual syndrome characterized by fever, hepatosplenomegaly, cytopenias, hypertriglyceridemia, hypofibrinogenemia, and pathologic findings of hemophagocytosis in the bone marrow and other tissues. HLH may be familial or associated with different types of infections, autoimmune disorders, or malignancies. Infection-associated HLH has been reported i...

The term macrophage activation syndrome (MAS) identifies a severe and potentially fatal complication of s-JIA, and, more rarely of other rheumatic diseases. MAS share similarities in clinical features and laboratory abnormalities with primary and secondary heamophagocytic lymphohystiocytosis (HLH). Indeed it is currently classified among secondary HLH and the term rheuma-HLH has been used to in...

Hemophagocyticlymphohistiocytosis (HLH) is a hyper inflammatory disorder which results from immune dysfunction, either from primary immune deficiency or acquired failure of normal immune homeostasis. There is often phagocytosis by macrophages of blood cells, hence the name “hemophagocytosis” [1]. HLH may be inherited (known as familial hemophagocyticlymphohistiocytosis), or it may occur seconda...

Hemophagocytic lymphohistiocytosis (HLH) is a critical condition that may lead to organ failure and early death. The aim of this retrospective observational study was to describe a cohort of HLH patients admitted to intensive care unit (ICU) and investigate the risk factors of early death. A positive HLH diagnosis was defined by an HScore 169. Univariate and multivariate analyses were carried o...

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome characterized by overwhelming immune activation. A steroid and chemotherapy-based regimen remains as the first-line of therapy but it has substantial morbidity. Thus, novel, less toxic therapy for HLH is urgently needed. Although differences exist between familial HLH (FHL) and secondary HLH (sHLH), they have many common fe...

Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal hyperinflammatory condition. It may occur as a primary (genetic) condition due to mutations in genes important in the cytolytic secretory pathway that cause perforin and granzymes to induce apoptosis in target cells. Primary HLH is divided into familial HLH (FHLH1-5), in which HLH is the only manifestation of disease, and other gen...

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening, virus-triggered immune disease. Hypersensitivity to mosquito bite (HMB), a presentation of Chronic Active Epstein-Barr Virus infection (CAEBV), may progress to HLH. This study aimed to investigate the immunologic difference between the HMB episodes and the HLH episodes associated with EBV infection. Immunologic changes of immunoglo...