Premium
Hematopoietic stem cell transplantation for children with primary immunodeficiency diseases: Single center experience in Jordan
Author(s) -
Amayiri Nisreen,
AlZaben Abdulhadi,
Ghatasheh Lubna,
Frangoul Haydar,
Hussein Ayad Ahmed
Publication year - 2013
Publication title -
pediatric transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.457
H-Index - 69
eISSN - 1399-3046
pISSN - 1397-3142
DOI - 10.1111/petr.12081
Subject(s) - medicine , hematopoietic stem cell transplantation , primary immunodeficiency , pediatrics , single center , digeorge syndrome , transplantation , surgery , tuberculosis , disease , pathology , psychiatry
HSCT can be curative for many PID . Little is known about the outcome of HSCT for patients with PID in the developing countries. We retrospectively reviewed all children with PID who received HSCT at KHCC in J ordan between A ugust 2003 and O ctober 2011. Twenty‐eight patients were identified. The median age was 16 months (3 months–17 yr). Patients' diagnoses were SCID (n = 16), CHS (n = 3), HLH (n = 3), WAS (n = 2), G riscelli syndrome (n = 1), ALPS (n = 1), O menn's syndrome (n = 1), and DiGeorge syndrome (n = 1). Seventeen patients received HLA ‐matched related HSCT , eight received maternal un‐manipulated haploidentical HSCT , and three received unrelated cord blood transplantation. Nine patients (32%) developed BCG osis secondary to reactivation of pretransplant vaccination. Three died while still receiving anti‐tuberculosis drugs, one still on treatment, and all others have recovered. Six patients had graft failure; four of them received no conditioning regimens. At a median follow up of 32 months (range 1‐67), 21 patients are alive, with overall survival of 72%. We conclude that HSCT for PID patients can be performed with a good outcome in developing countries; however, delayed diagnosis or referral and BCG reactivation are unique challenges.