hemophagocytic lymphohistiocytosis (hlh) is an aggressive and potentially life-threatening disease and has to be considered in the differential diagnosis of many conditions. hlh comprises two different conditions that are difficult to differentiate; familial hemophagocytic lymphohistiocytosis (fhlh) or familial erythrophagocytic lymphohistiocytosis (fel), and secondary hemophagocytic syndromes ...

Familial haemophagocytic lymphohistiocytosis is a rare but invariably fatal disease without haematopoietic stem cell transplantation. Genetic defect identification is useful for confirming a clinical diagnosis, predicting the risk of future recurrence, and defining haemophagocytic lymphohistiocytosis predisposition in asymptomatic family members. Notably, familial haemophagocytic lymphohistiocy...

Introduction Hemophagocytic syndrome or hemophagocytic lymphohistiocytosis is a rare condition characterized by excessive inflammation that is thought to be caused by the absence of normal downregulation of activated macrophages and lymphocytes. The treatment of hemophagocytic lymphohistiocytosis can depend on whether it is primary or secondary. In secondary hemophagocytic lymphohistiocytosis, ...

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. ...

BACKGROUND Hemophagocytic lymphohistiocytosis in adults is often secundary to an infection or a neoplasm. In this last case, T cell lymphomas are the most frequent causes. Hemophagocytic lymphohistiocytosis secundary to a B cell lymphoma has been rarely reported. CASE PRESENTATION We describe a case of a hemophagocytic lymphohistiocytosis complicating a T-cell rich B-cell lymphoma treated wit...

OBJECTIVE To study the clinical profile of children with scrub typhus and its association with hemophagocytic lymphohistiocytosis. METHODS Children presenting with unexplained fever and multi-systemic involvement between May to December 2011 were tested for scrub typhus using IgM ELISA kits. Occurrence of Hemophagocytic lymphohistiocytosis in IgM positive cases of scrub typhus was studied. ...

BACKGROUND Both haemophagocytic lymphohistiocytosis and acute necrotizing encephalopathy are life-threatening condition. It presents major diagnostic difficulties, since it may have a diversity in clinical picture and with many conditions leading to the same clinical presentation. So it is key important to understand the disorders. CASE PRESENTATION We report a pediatric case of haemophagocyt...

Hemophagocytic lymphohistiocytosis is a rare and often fatal disease that may occur in solid organ transplant recipients. Here, we describe 2 patients who developed hemophagocytic lymphohistiocytosis after having a lung transplant and present a review of all cases of hemophagocytic lymphohistiocytosis occurring in solid organ transplant recipients. Diagnosis of hemophagocytic lymphohistiocytosi...

Scrub typhus is caused by Orientia tsutsugamushi. Any delay in diagnosis can result in delayed treatment and severe complications, including secondary hemophagocytic lymphohistiocytosis, which is rare but potentially fatal. In this paper, the authors present 3 cases of secondary hemophagocytic lymphohistiocytosis associated with scrub typhus, successfully treated with chloramphenicol without ad...

BACKGROUND Hemophagocytic lymphohistiocytosis associated with autoimmune diseases is seen in patients with systemic juvenile idiopathic arthritis, adult-onset Still's disease, and systemic lupus erythematosus, whereas it is rarely seen in patients with dermatomyositis. In addition, central nervous system involvement with dermatomyositis is rare. To the best of our knowledge, this is the first c...