hemophagocytic lymphohistiocytosis (hlh) is one of the complications of epstein-barr virus (ebv) infection. although the patients who have developed hlh following ebv have normal immune system, there are a few patients with ebv-induced immune deficiency who develop hlh as well. here, we describe the case of a 10-year-old girl with neurological complications caused by ebv-induced hlh. the patien...

Adult listeners rated the difficulty of hearing a single coherent stream in a sequence of high (H) and low (L) tones that alternated in a repetitive galloping pattern (HLH-HLH-HLH...). They could hear the gallop when the sequence was perceived as a single stream, but when it segregated into two substreams, they heard H-H- ... in one stream and L-L- ... in the other. The onset-to-onset time of t...

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...

Autoimmune lymphoproliferative syndrome (ALPS) is a rare inherited disorder of apoptosis, most commonly due to mutations in the FAS (TNFRSF6) gene. It presents with chronic lymphadenopathy, splenomegaly, and symptomatic multilineage cytopenias in an otherwise healthy child. Unfortunately, these clinical findings are also noted in other childhood lymphoproliferative conditions, such as leukemia,...

Mutations in the perforin gene (PRF1) are a common cause of the fatal immune dysregulation disorder, familial hemophagocytic lymphohistiocytosis (type 2 FHL, FHL2). Here we report a female infant born with biallelic PRF1 mutations: a novel substitution, D49N, and a previously identified in-frame deletion, K285del. We assessed the effects of each mutation on the cytotoxicity of human NK cells in...

Hemophagocytic Lymphohistiocytosis (HLH) implies a benign generalized histiocytic proliferate with erythrophagocytosis and it includes familial hemophagocytic lymphohistiocytosis and secondary hemophgocytosis. Spinal fluid changes like mild to moderate pleocytosis (most of the cells are lymphocytes and macrophages) and sometimes hemophagocytosis are seen in primary HLH but are not reported in s...

Hemophagocytic lymphohistocytosis (HLH) is a hyperinflammatory syndrome that occurs at all ages and is characterized by high levels of cytokines, secreted by activated T-lymphocytes and macrophages. All symptoms and laboratory changes can be explained by organ infiltration by these cells and hypercytokinemia. HLH occurs as an inherited form (genetic, primary HLH) with mutations primarily in the...

BACKGROUND Hemophagocytic lymphohistiocytosis is an aggressive life-threatening syndrome of excessive immune activation. Hemophagocytic lymphohistiocytosis due to systemic lupus erythematosus is described as acute lupus hemophagocytic syndrome. Acute lupus hemophagocytic syndrome presenting with negative antinuclear antibody is uncommon. CASE PRESENTATION A 57-year-old Sri Lankan woman presen...