BACKGROUND Hemophagocytic lymphohistiocytosis is a life-threatening disease. Hematopoietic stem cell transplantation still represents the treatment of choice for most patients with this disease. DESIGN AND METHODS We retrospectively analyzed 61 patients with hemophagocytic lymphohistiocytosis who underwent HSCT over a 17-year period at nine centers affiliated to the Italian Pediatric Hematolo...

Hemophagocytic lymphohistiocytosis (HLH) or hemophagocytic syndrome is a life threatening hyperinflammatory condition caused by immune dysregulation and resulting in hemophagocytosis and organ damage by activated macrophages and histiocytes. The characteristic clinical features include fever, splenomegaly, cytopenias, liver dysfunction, and hyperferritinemia. HLH can be either primary (genetic)...

Dengue, transmitted by the mosquito Aedes aegypti affects millions of people worldwide every year. Dengue induced hemophagocytic lymphohistiocytosis (HLH) is a serious condition and may prove fatal if not detected early and treated appropriately. Diagnosis of HLH is challenging and usually missed as clinical and laboratory findings are nonspecific. Moreover, the pathophysiology of the systemic ...

BACKGROUND Hemophagocytic lymphohistiocytosis is a frequently fatal and likely underdiagnosed disease. It is a rare occurrence in adults and usually secondary to an insult such as viral infections, bacterial infections, autoimmune connective tissue disorders, malignancies and immunocompromised states, in contrast to its childhood counterpart, which is due to a genetic defect but may share some ...

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of excessive immune activation causing widespread inflammation and tissue destruction leading to multi-organ dysfunction and failure. Making the diagnosis of HLH could be quite challenging due to the broad range of presenting symptoms and their lack of specificity. After ruling out considerations for differential diagnoses, recognizing the ...

BACKGROUND Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening hyperinflammatory/hypercytokinemia syndrome clinicopathologically manifested by fever, hepatosplenomegaly, cytopenias, liver dysfunction, and hemophagocytosis. METHODS We searched the medical literature for English-written articles and analyzed data regarding the diagnosis, pathoetiology, prognosis, and managem...

Hemophagocytic lymphohystiocytosis (HLH) represents a severe hyperinflammatory condition with the cardinal symptoms prolonged fever, hepatosplenomegaly, and cytopenias. The most prominent histopathological feature of HLH is an accumulation of activated T lymphocytes and macrophages predominantly in lymphoid tissues. Although it can occur in all age groups, neonatal-onset HLH is very rare. We re...

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of pathologic immune activation, occurring as either a familial disorder or a sporadic condition, in association with a variety of triggers. This immune dysregulatory disorder is prominently associated with cytopenias and a unique combination of clinical signs and symptoms of extreme inflammation. Prompt initiation of immunochemotherapy is ...

Hemophagocytic lymphohistiocytosis (hemophagocytic syndrome, HLH, HPS) is the group of severe life-threatening and hardly diagnosing conditions caused by immune dysregulation because systemic inflammatory response with non-controlled proliferation activation T-cells, monocytes macrophages accumulation in target organs development multiple organ failure. HLH are includes primary (monogenic) seco...

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...