HLH was first described by Farquhar and Claireaux in 1952 as a familial disease [1]. Interestingly in their cases hemophagocytosis, which has given the disease its name, could not be found during lifetime but was prominent on autopsy. Familial hemophagocytic lymphohistiocytosis (FHLH) is an autosomal recessive disorder with an estimated frequency of 0.12/100 000 children per year [2]. Several g...

Hemophagocytic Lymphohistiocytosis (HLH) is a rare, lifethreatening syndrome of excessive multisystem inflammation and tissue destruction due to abnormal immune activation. It is more frequently seen in the pediatric population, either as a genetic disorder or secondary to infection or autoimmune disease, but can present in acquired forms at any age. It is thought to be due to an absence of dow...

In this case we discussed with a hemaphagocytic syndrome in patient who was investigated for jaundice. Hemophagocytic syndrome; fever, hypertriglyceridemia, hepatosplenomegaly, bone marrow involvement is multisystemic disease.This due to excess cytokines released from the cells.Hemophagocytic syndrome, can occur as familial or sporadic disorder, and it be triggered by variety of events that dis...

This is a case report with serial imaging showing progression of diffuse osteonecrosis in a patient after a diagnosis of secondary hemophagocytic lymphohistiocytosis (HLH). While bone marrow involvement in HLH has been long noted at histological evaluation and is itself one of the diagnosis criteria, to the best of our knowledge, there has been no previous publication addressing osseous image f...

In four experiments, the accumulation, over time, of a tendency to hear separate high and low streams in a sequence of high (H) and low (L) tones, presented in a galloping rhythm (HLH-HLH-...), was studied. Each trial was composed of two parts, an induction sequence, then a test sequence, with no break between them. The test sequence was always heard at the far left. When the induction sequence...

Macrophage activation syndrome (MAS) is a rare feature of rheumatic disorders in children and adolescence and its presentation as the first symptom of rheumatic disorders is very infrequent. A 9-year-old girl, in whom MAS developed, was admitted to our Hospital in Tehran, Iran. She suffered from high grade fever and rash followed by multiple joint swelling months afterwards. Bone marrow aspira...