primary immunodeficiencies (pid) are a group of disorders, characterized by an unusual susceptibility to infections. delay in diagnosis results in increased morbidity and mortality in affected patients. the purpose of this study was to determine the mortality rate of iranian immunodeficient patients referred to children medical center hospital affiliated to tehran university of medical sciences...

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...

epstein-barr virus (ebv) is a ubiquitous human -herpesvirus that infects about 95% of the adult population. the majority of primary infections occurs in early childhood and is generally subclinical; it can cause infectious mononucleosis (im), which is usually a self-limiting lymphoproliferative disorder. however, infection of ebv occasionally results in severe, often lethal diseases, which inc...

autoimmune lymphoproliferative syndrome (alps) is an uncommon nonmalignant lymphoproliferative disease which is characterized by chronic, persistent or recurrent lymphadenopathy, splenomegaly, hepatomegaly, immune cytopenia, hypergammaglobinemia and increased risk of lymphoma. we report a 2-year old boy with hepatosplenomegaly as first presentation. petechial and purpuric rashes with massive ce...