An overwhelming activation of cytotoxic T cells and well-differentiated macrophages leading
to systemic overload of inflammatory mediators characterizes the so-called macrophage …

Background Recently, there have been increasingly frequent reports on the occurrence of
macrophage activation syndrome (MAS) in patients with inflammatory bowel disease (IBD) …

Hemophagocytic lymphohistiocytosis is a hyperinflammatory syndrome defined by clinical
and laboratory criteria. Current criteria were created to identify patients with familial …

An array of monogenic immune defects marked by autoimmunity, lymphoproliferation, and
hyperinflammation rather than infections have been described. Primary immune regulatory …

Aim: To report a case of a female patient with hemophagocytic lymphohistiocytosis (HLH)
and to systematically review the available cases of the association between HLH and …

Paroxysmal crises of fever and systemic inflammation herald familial Mediterranean fever
(FMF), considered as the archetype of all inherited systemic autoinflammatory diseases …

Background Hemophagocytic Lymphohistiocytosis (HLH) is a rare and life-threatening
condition characterized by a severe impairment of the immune homeostasis. While Familial …

Familial Mediterranean fever (FMF) is the most common and best known of hereditary
recurrent fever or periodic fever syndromes. It was described in 1945 and genetically …

A chest film revealed bilateral infiltrates (Fig. 1A) and an ultrasound evaluation of the
abdomen was negative except for a slightly enlarged spleen. Blood and urine cultures as …

Patients with inflammatory bowel disease (IBD) are at risk of developing haemophagocytic
lymphohistiocytosis (HLH) because of chronic systemic inflammation as well as exposure to …