Hematopoietic stem cell transplantation for children with primary immunodeficiency diseases: single center experience in Jordan. 2013

Nisreen Amayiri, and Abdulhadi Al-Zaben, and Lubna Ghatasheh, and Haydar Frangoul, and Ayad Ahmed Hussein
Department of Pediatrics, KHCC, Amman, Jordan.

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 Jordan between August 2003 and October 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), Griscelli syndrome (n = 1), ALPS (n = 1), Omenn'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 BCGosis 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.

UI MeSH Term Description Entries
D007153 Immunologic Deficiency Syndromes Syndromes in which there is a deficiency or defect in the mechanisms of immunity, either cellular or humoral. Antibody Deficiency Syndrome,Deficiency Syndrome, Immunologic,Deficiency Syndromes, Antibody,Deficiency Syndromes, Immunologic,Immunologic Deficiency Syndrome,Immunological Deficiency Syndromes,Antibody Deficiency Syndromes,Deficiency Syndrome, Antibody,Deficiency Syndrome, Immunological,Deficiency Syndromes, Immunological,Immunological Deficiency Syndrome,Syndrome, Antibody Deficiency,Syndrome, Immunologic Deficiency,Syndrome, Immunological Deficiency,Syndromes, Antibody Deficiency,Syndromes, Immunologic Deficiency,Syndromes, Immunological Deficiency
D007223 Infant A child between 1 and 23 months of age. Infants
D007597 Jordan A country in the MIDDLE EAST, northwest of Saudi Arabia, between Israel and Iraq. The capital is Amman.
D008297 Male Males
D010859 Pigmentation Disorders Diseases affecting PIGMENTATION, including SKIN PIGMENTATION. Incontinentia Pigmenti Achromians,Ito Syndrome,Schamberg's Disease,Schamberg Disease,Disease, Schamberg,Disease, Schamberg's,Disorder, Pigmentation,Disorders, Pigmentation,Pigmentation Disorder,Schambergs Disease,Syndrome, Ito
D002609 Chediak-Higashi Syndrome A form of phagocyte bactericidal dysfunction characterized by unusual oculocutaneous albinism, high incidence of lymphoreticular neoplasms, and recurrent pyogenic infections. In many cell types, abnormal lysosomes are present leading to defective pigment distribution and abnormal neutrophil functions. The disease is transmitted by autosomal recessive inheritance and a similar disorder occurs in the beige mouse, the Aleutian mink, and albino Hereford cattle. Chediak-Steinbrinck-Higashi Syndrome,Oculocutaneous Albinism with Leukocyte Defect,Chediak Higashi Syndrome,Chediak Steinbrinck Higashi Syndrome,Chediak-Steinbrinck-Higashi Syndromes
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D004062 DiGeorge Syndrome Congenital syndrome characterized by a wide spectrum of characteristics including the absence of the THYMUS and PARATHYROID GLANDS resulting in T-cell immunodeficiency, HYPOCALCEMIA, defects in the outflow tract of the heart, and craniofacial anomalies. Velocardiofacial Syndrome,22q11.2 Deletion Syndrome,22q11.2DS,Autosomal Dominant Opitz G-Bbb Syndrome,Catch22,Conotruncal Anomaly Face Syndrome,Conotruncal Anomaly Face Syndrome (CTAF),Deletion 22q11.2 Syndrome,DiGeorge Anomaly,DiGeorge Sequence,Familial Third and Fourth Pharyngeal Pouch Syndrome,Hypoplasia of Thymus and Parathyroids,Pharyngeal Pouch Syndrome,Sedlackova Syndrome,Shprintzen Syndrome,Shprintzen VCF Syndrome,Third and Fourth Pharyngeal Pouch Syndrome,Thymic Aplasia Syndrome,VCF Syndrome,Velo-Cardio-Facial Syndrome,Autosomal Dominant Opitz G Bbb Syndrome,Deletion Syndrome, 22q11.2,Syndrome, DiGeorge,Syndrome, Sedlackova,Syndrome, Shprintzen,Syndrome, VCF,Syndrome, Velo-Cardio-Facial,Syndrome, Velocardiofacial,Velo Cardio Facial Syndrome
D005260 Female Females

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