A 22-year-old man was admitted to our hospital because of fever, skin rash and epistaxis. Physical examination revealed fever (39.5°C), generalized purpura, lymphadenopathy and splenomegaly. Blood tests showed pancytopenia. Bone marrow aspiration and biopsy showed hemophagocytosis with no evidence of malignant cells. Anti rubella IgM antibody were positive and the IgG titers increased from 16 t...

UNLABELLED Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening condition. Uncontrolled proliferation of activated lymphocytes secreting high amounts of inflammatory cytokines seems to be the main pathogenesis. The diagnosis of HLH can often be difficult. It may presents in many forms such as fever of unknown origin, hepatitis, acute liver failure, and sepsis-like illness. Here w...

Mild anemia and leukopenia are the most common hematologic problems of acute brucellosis. Mild thrombocytopenia also occurs, but severe cases are uncommon. Thrombocytopenia occurs because of bone marrow suppression, hypersplenisem, hemophagocytosis, and immunologic destruction of the cells or disseminated intravascular coagulation. In endemic areas, hemorrhagic fevers, hematologic malignancies,...

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 ...

11 Summary Despite the fact that anemia is one of the most striking clinical features of visceral leishmaniasis (kala-azar), the factors involved in the pathogenesis are not fully understood. The cause of anemia seen in these patients is often multifactorial including sequestration and destruction of the erythrocytes in the enlarged spleen, hemophagocytosis and alterations in erythrocyte membra...

Familial Hemophagocytic lymphohistiocytosis (FHL) is a fatal autosomal recessive hyperinflammatory syndrome characterized by fever, hepatosplenomegaly, cytopenia, hyperferritenemia and widespread hemophagocytosis in the reticuloendothelial tissues. Diagnosis of FHL can be confirmed by the presence of typical clinical and laboratory features along with presence of a specific genetic mutation. Mu...

Cytophagic histiocytic panniculitis is a rare disease, associated with either nonmalignant conditions or subcutaneous panniculitis-like T-cell lymphoma, and often also associated with hemophagocytic lymphohistiocytosis (HLH). We report the case of a 11-year-old boy with a history of secondary HLH who, after a local trauma, developed a painful, indurated plaque over the right thigh associated wi...