PMID- 24231474
OWN - NLM
STAT- MEDLINE
DCOM- 20140116
LR  - 20131115
IS  - 1319-2442 (Print)
IS  - 1319-2442 (Linking)
VI  - 24
IP  - 6
DP  - 2013 Nov
TI  - Mismatched related hematopoietic stem cell transplantation in primary 
      immunodeficiency.
PG  - 1137-43
LID - 10.4103/1319-2442.121269 [doi]
AB  - Hematopoietic stem cell transplantation (HSCT) is the definitive therapy for a 
      variety of primary immunodeficiency syndromes (PIDs). However, no more than 30% 
      of the patients will have a human leukocyte antigen (HLA)-identical sibling. We 
      retrospectively analyzed our results of ten patients with PID; severe combined 
      immunodeficiency (SCID) (n = 7), hyper IgM (HIgM) (n = 1) and combined 
      immunodeficiency (CID) (n = 2), who lacked a fully matched donor and underwent 
      mismatched related HSCT during the period from 2008 to 2010. The median age at 
      the time of transplantation ranged between 3 and 84 months (median 6.5 months). 
      Peripheral blood stem cells (PBSC) were used in all HSCTs. The mean value of the 
      peripheral CD34+ cells infused was 9.19 x 10 (6) /kg recipient weight. Patients 
      received different conditioning protocols. All patients received anti graft 
      versus host disease (GVHD) prophylaxis and all were engrafted. Mixed chimerism 
      (5-55%) was noticed. GVHD was observed in 50% of the patients. Post-transplant 
      follow-up ranged from 3 weeks to 36 months (median 15 months). Five patients are 
      still alive while one patient developed engraftment syndrome followed by graft 
      slippage for which a second transplant with CD34+ stem cells 5.8 x 10 (6) /kg 
      recipient's weight was infused. The others died from sepsis and 
      transplant-related complications. Immune reconstitution was noticed in four 
      patients. In conclusion, HLA-haploidentical stem cell transplantation may be 
      feasible, with appropriate GVHD prophylaxis, for patients with PID who lack a 
      fully matched donor.
FAU - Wahadneh, Adel M
AU  - Wahadneh AM
AD  - Queen Rania Children Hospital, King Hussein Medical Center, Royal Medical 
      Services, Amman, Jordan.
FAU - Bin Dahman, Haifa A
AU  - Bin Dahman HA
FAU - Abu Shukear, Mohammed E
AU  - Abu Shukear ME
FAU - Habahbeh, Zeyad M
AU  - Habahbeh ZM
FAU - Ajarmeh, Mohammad A
AU  - Ajarmeh MA
FAU - Zyood, Raed M
AU  - Zyood RM
FAU - Habashneh, Mueen S
AU  - Habashneh MS
LA  - eng
PT  - Journal Article
PL  - Saudi Arabia
TA  - Saudi J Kidney Dis Transpl
JT  - Saudi journal of kidney diseases and transplantation : an official publication of 
      the Saudi Center for Organ Transplantation, Saudi Arabia
JID - 9436968
SB  - IM
MH  - Child
MH  - Child, Preschool
MH  - Female
MH  - Graft vs Host Disease/prevention & control
MH  - *Hematopoietic Stem Cell Transplantation
MH  - Histocompatibility/*immunology
MH  - Humans
MH  - Infant
MH  - Male
MH  - Retrospective Studies
MH  - Severe Combined Immunodeficiency/*surgery
MH  - Transplantation Conditioning
EDAT- 2013/11/16 06:00
MHDA- 2014/01/17 06:00
CRDT- 2013/11/16 06:00
PHST- 2013/11/16 06:00 [entrez]
PHST- 2013/11/16 06:00 [pubmed]
PHST- 2014/01/17 06:00 [medline]
AID - SaudiJKidneyDisTranspl_2013_24_6_1137_121269 [pii]
AID - 10.4103/1319-2442.121269 [doi]
PST - ppublish
SO  - Saudi J Kidney Dis Transpl. 2013 Nov;24(6):1137-43. doi: 
      10.4103/1319-2442.121269.