INTRODUCTION Hemophagocytic syndrome is patophysiological entity with proliferation and over-activation of macrophages, with hemophagocytosis and production of proinflammatory cytokines. It arises as hereditary forms, or acquired, during viral, autoimmune or malignant diseases, and is usually a disorder with fulminant course and high incidence of lethal outcome. The precise mechanism is not res...

Hemophagocytic syndrome (HPS) is described by an increase in macrophages accountable for extensive phagocytosis of hematopoietic cells. Secondary HPS arises commonly in the presence of infections, neoplasia, autoimmune disorders and immune disorders. Here, we reported a patient with common variable immune deficiency (CVID) and Hodgkin’s lymphoma (HL) who later developed EBV linked hemophagocyti...

Hemophagocytic lymphohistiocytosis (HLH) is an immune dysregulation syndrome which is characterized by widespread but ineffective activation of immune system of our body. This activation leads to release of a large pool of cytokines from the activated lymphocytes and macrophages. This hypercytokinemia leads to the development of characteristic features of HLH such as fever, cytopenias, hepatosp...

INTRODUCTION Major hyperferritinemia is a rare feature in clinical laboratories associated with a wide variety of disorders, including hemophagocytic lymphohistiocytosis (HLH). The diagnosis of HLH is based on clinical and biological criteria, such as those proposed by the Histiocyte Society. However, several of these criteria are not relevant in the specific setting of hematologic malignancies...

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 article will introduce a neonate with hlh in iran. we report a case of hlh presenting with respiratory distress and fever, hepatosplenomegaly, jaundice and pancytopenia on the second day of life. ...