title: Successful fludarabine-based hematopoietic stem cell transplantation in a pediatric patient with idiopathic CD4+ lymphocytopenia.
abstract: Idiopathic CD4+ lymphocytopenia (ICL) is a rare immunodeficiency disease with severe CD4 T-cell depletion, leading to serious opportunistic infections. The optimal treatment of ICL has not been determined, especially in severe form of the disease. Here, we report an eight-yr-old girl with ICL who was successfully treated with fludarabine-based conditioning HSCT. To the best of our knowledge, this is the first pediatric ICL case that was treated by HSCT. Allogeneic HSCT with a reduced intensity condition (RIC) regimen may be a feasible and curative treatment option in ICL patients with recurrent life-threatening complications.
authors.affiliation: Hematology-Oncology and Stem Cell Transplantation Research Center, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: Amir Ali
authors.initials: AA
authors.lastname: Hamidieh
authors.forename: Zahra
authors.initials: Z
authors.lastname: Pourpak
authors.forename: Amir
authors.initials: A
authors.lastname: Hamdi
authors.forename: Mohammad
authors.initials: M
authors.lastname: Nabavi
authors.forename: Ardeshir
authors.initials: A
authors.lastname: Ghavamzadeh
mesh_terms: D015496:CD4-Positive T-Lymphocytes / Q000166:cytology*; D002648:Child; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation / Q000379:methods*; D006801:Humans; D008231:Lymphopenia / Q000628:therapy*; D019653:Myeloablative Agonists / Q000627:therapeutic use*; D009894:Opportunistic Infections / Q000517:prevention & control; D019172:Transplantation Conditioning / Q000379:methods*; D016896:Treatment Outcome; D014740:Vidarabine / Q000031:analogs & derivatives* / Q000627:therapeutic use
chemical_list: D019653:Myeloablative Agonists; D014740:Vidarabine; C024352:fludarabine