title: Primary hemophagocytic lymphohistiocytosis in Iran: report from a single referral center.
abstract: Hemophagocytic lymphohistiocytosis (HLH) is a rare condition characterized by fever, hepatosplenomegaly, and cytopenia, and widespread accumulation of lymphocytes and histiocytes, sometimes with hemophagocytosis, primarily involving the spleen, lymph nodes, bone marrow, and liver. HLH can either occur sporadically (secondary HLH) or as part of a familial syndrome (primary HLH), including familial HLH and the distinct immunodeficiency syndromes. Herein the authors report 6 Iranian patients with primary HLH and their outcome from a single tertiary-care center.
authors.affiliation: Department of Pediatric Hematology-Oncology, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran. Shamisianb@yahoo.com
authors.forename: Bibi Shahin
authors.initials: BS
authors.lastname: Shamsian
authors.forename: Nima
authors.initials: N
authors.lastname: Rezaei
authors.forename: Samin
authors.initials: S
authors.lastname: Alavi
authors.forename: Mona
authors.initials: M
authors.lastname: Hedayat
authors.forename: Ali
authors.initials: A
authors.lastname: Amin Asnafi
authors.forename: Zahra
authors.initials: Z
authors.lastname: Pourpak
authors.forename: Atoosa
authors.initials: A
authors.lastname: Gharib
authors.forename: Farzaneh
authors.initials: F
authors.lastname: Jadali
authors.forename: Mohammad Taghi
authors.initials: MT
authors.lastname: Arzanian
mesh_terms: D005190:Family; D006801:Humans; D007492:Iran; D051359:Lymphohistiocytosis, Hemophagocytic / Q000235:genetics* / Q000628:therapy; D009154:Mutation; D016896:Treatment Outcome; D020691:rab GTP-Binding Proteins / Q000235:genetics; D000075745:rab27 GTP-Binding Proteins
chemical_list: D000075745:rab27 GTP-Binding Proteins; C066694:RAB27A protein, human; D020691:rab GTP-Binding Proteins