Hemophagocytic syndrome (HPS), also known as hemophagocytic lymphohistiocytosis (HLH) encompasses an infrequent group of non-malignant, yet potentially life-threatening disorders caused by massive cytokine release from activated lymphocytes and macrophages (Filipovich, 2009; Henter et al., 1998, 2007; Janka et al., 1998; Janka, 2009). This multisystem inflammatory syndrome is associated with a ...

Hemophagocytic syndrome is a non-malignant process that is characterized clinically by fever, hepatomegaly, splenomegaly, pancytopenia in peripheral blood, and reactive histiocytes in the bone marrow. Bacterial infectious diseases like typhoid fever and brucellosis and viral infections including CMV, herpes viruses, and Epstein-Barr virus are diagnosed as the cause of this syndrome. In thi...

hemophagocytic syndrome is a non-malignant process that is characterized clinically by fever, hepatomegaly, splenomegaly, pancytopenia in peripheral blood, and reactive histiocytes in the bone marrow. bacterial infectious diseases like typhoid fever and brucellosis and viral infections including cmv, herpes viruses, and epstein-barr virus are diagnosed as the cause of this syndrome. in this pap...

Histiocytic syndromes in children are divided into 3 classes: (i) Langerhans-cell histiocytosis (LCH) or Histiocytosis-X (HX), (ii) Hemophagocytic syndromes—Familial erythrophagocytic lymphohistiocytosis (FEL) and infection-associated hemophagocytic syndrome (IAHS) due to viruses (VAHS), and occasionally bacterial, fungal or parasitic, and (iii) Malignant histiocytic disorders(l). The lesional ...

Background Early diagnosis of macrophage activations syndrome (MAS) in systemic juvenile idiopathic arthritis (sJIA) may be challenging because it may mimic the clinical features of the underlying disease or be confused with an infectious complication. However, the diagnostic value of the guidelines for hemophagocytic lymphohistiocytosis (HLH) (1) or sJIA-associated MAS (2) has seldom been exam...

Mutations of familial hemophagocytic lymphohistiocytosis (FHL) related genes and abnormalities of cytotoxicity function tests in patients with macrophage activation syndrome (MAS) occurring in systemic juvenile idiopathic arthritis (sJIA) Claudia Bracaglia, Elena Sieni, Martina Da Ros, Carmela De Fusco, Concetta Micalizzi, Valentina Cetica, Benedetta Ciambotti, Maria Luisa Coniglio, Antonella I...

Macrophage activation syndrome (MAS), also known as secondary hemophagocytic lymphohistiocytosis, is mediated by cytokine overproduction from excessive activation of T lymphocytes and macrophages. We present a dermatomyositis patient with MAS, caused by hypercytokinemia. The combination of tacrolimus and plasma exchange therapy was effective in this case for treating MAS. This combination thera...

INTRODUCTION Macrophage activating syndrome is a severe, potentially life-threatening condition that may accompany Still's disease. It is characterized by fever, hepatosplenomegaly, lymphadenopathy, severe cytopenia, serious liver dysfunction, coagulopathy and neurologic involvement. The principal treatment for patients with this syndrome includes etoposide 150 mg/2 M twice a week for two weeks...

INTRODUCTION Hemophagocytic lymphohistiocytosis induced by viral diseases is a well recognized entity. Severe forms of H5N1 influenza are known to be associated with symptoms very similar to a reactive hemophagocytic syndrome. We report a case of fulminant lymphohistiocytosis associated with the pandemic A (H1N1) variant. CASE PRESENTATION A 42-year-old Caucasian woman developed a syndrome of...

Introduction: The people worldwide have been affected by severe acute respiratory syndrome-coronavirus 2 (SARS-CoV-2) infection since its appearance in December, 2019. Kawasaki disease-like hyperinflammatory shock associated with SARS-CoV-2 previously healthy children has reported the literature, which is now referred to as a multisystem inflammatory syndrome (MIS-C). Some aspects of MIS-C are ...