PMID- 33628130
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20220420
IS  - 1512-7680 (Print)
IS  - 1986-597X (Electronic)
IS  - 1512-7680 (Linking)
VI  - 32
IP  - 4
DP  - 2020 Dec
TI  - Hematopoietic Stem Cell Transplantation in Thalassemia Patients: a Jordanian 
      Single Centre Experience.
PG  - 277-282
LID - 10.5455/msm.2020.32.277-282 [doi]
AB  - INTRODUCTION: Beta thalassemia major is the commonest inherited hematological 
      disorder worldwide which needs lifelong sufficient supportive management. 
      Hematopoietic stem Cell transplantation (HSCT) is the only curative treatment 
      available till now. AIM: To evaluate the outcome of children who underwent 
      allogenic hematopoietic stem Cell transplantation as a curative approach for 
      Thalassemia Major, treated at Queen Rania AL- Abdullah children Hospital (QRCH). 
      METHODS: A retrospective review of the medical files was conducted for all 
      children (< 15 years) who had thalassemia major and received HSCT between 
      January, 2010 and January, 2019. The following variables were studied for all 
      patients: age , gender, Pesaro classifications, the count of infused raw bone 
      marrow stem cell (CD34), engraftment time, outcome and complications. RESULTS: A 
      total of 34 children were transplanted for thalassemia major, at an average of 4 
      cases per year. All underwent allogenic raw bone marrow transplantation from 
      matched related donors. Thirteen patients (38.2%) were males and twenty one 
      (61.2%) were females. The age ranged between 2 and 15 years, with a median age of 
      6.5 years. According to Pesaro classification, 31 patients were class 2 (91.2%) 
      and 3 patients were class 3 (8.8%) while no single case met the criteria for 
      class 1 Pesaro classification. The median CD34 count was 3.5 million/Kg of 
      recipient weight (range, 1.5*10(6)-7*10(6) /kg). The median time for neutrophil 
      engraftment was 15.5 days. At a median follow up of 5 years (range 1- 9.5), 33 
      patients were alive. One patient died before 100 days post transplantation due to 
      grade IV acute gastrointestinal Graft Versus Host Disease (GVHD). Three patients 
      had secondary graft failure (8.8%). Six patients (17.5 %) developed mild grade 
      1-2 skin GVHD while another patient developed hemorrhagic cystitis due to BK 
      virus and cytomegalovirus (CMV) which reactivated simultaneously, and was 
      successfully managed. CONCLUSION: The outlook for Thalassemia major has 
      dramatically changed after HSCT, with a considerable success in Jordan and 
      results comparable to international data.
CI  - (c) 2020 Maher Mustafa, Mousa Qatawneh, Mais Al Jazazi, Omaiema Jarrah, Ruba Al 
      Hazaimeh, Raida Oudat, Moath Al Tarawneh, Rami Al Majali.
FAU - Mustafa, Maher
AU  - Mustafa M
AD  - Department of Pediatric Hematology and Medical Oncology, Queen Rania Children 
      Hospital, Royal Medical Services, Amman, Jordan.
FAU - Qatawneh, Mousa
AU  - Qatawneh M
AD  - Department of Pediatric Hematology and Medical Oncology, Queen Rania Children 
      Hospital, Royal Medical Services, Amman, Jordan.
FAU - Al Jazazi, Mais
AU  - Al Jazazi M
AD  - Department of Pediatric Hematology and Medical Oncology, Queen Rania Children 
      Hospital, Royal Medical Services, Amman, Jordan.
FAU - Jarrah, Omaiema
AU  - Jarrah O
AD  - Department of Pediatric Hematology and Medical Oncology, Queen Rania Children 
      Hospital, Royal Medical Services, Amman, Jordan.
FAU - Al Hazaimeh, Ruba
AU  - Al Hazaimeh R
AD  - Department of Pediatric Hematology and Medical Oncology, Queen Rania Children 
      Hospital, Royal Medical Services, Amman, Jordan.
FAU - Oudat, Raida
AU  - Oudat R
AD  - Department of Hematopathology. Princess Iman Research and Laboratory Sciences 
      Centre, Royal Medical Services, Amman, Jordan.
FAU - Al Tarawneh, Moath
AU  - Al Tarawneh M
AD  - Department of Pediatric Hematology and Medical Oncology, Queen Rania Children 
      Hospital, Royal Medical Services, Amman, Jordan.
FAU - Al Majali, Rami
AU  - Al Majali R
AD  - Department of Pediatric Hematology and Medical Oncology, Queen Rania Children 
      Hospital, Royal Medical Services, Amman, Jordan.
LA  - eng
PT  - Journal Article
PL  - Bosnia and Herzegovina
TA  - Mater Sociomed
JT  - Materia socio-medica
JID - 101281595
PMC - PMC7879431
OTO - NOTNLM
OT  - Hematopoietic stem cell transplantation
OT  - Thalassemia
COIS- There are no conflicts of interest.
EDAT- 2021/02/26 06:00
MHDA- 2021/02/26 06:01
PMCR- 2020/12/01
CRDT- 2021/02/25 05:44
PHST- 2021/02/25 05:44 [entrez]
PHST- 2021/02/26 06:00 [pubmed]
PHST- 2021/02/26 06:01 [medline]
PHST- 2020/12/01 00:00 [pmc-release]
AID - MSM-32-277 [pii]
AID - 10.5455/msm.2020.32.277-282 [doi]
PST - ppublish
SO  - Mater Sociomed. 2020 Dec;32(4):277-282. doi: 10.5455/msm.2020.32.277-282.