PMID- 33472463
OWN - NLM
STAT- MEDLINE
DCOM- 20220602
LR  - 20220602
IS  - 1532-4311 (Electronic)
IS  - 0882-0139 (Linking)
VI  - 51
IP  - 4
DP  - 2022 May
TI  - Long Term Follow-Up of the Patients with Severe Combined Immunodeficiency After 
      Hematopoietic Stem Cell Transplantation: A Single-Center Study.
PG  - 739-747
LID - 10.1080/08820139.2020.1869776 [doi]
AB  - BACKGROUND: We aimed to evaluate hematopoietic stem cell transplantation (HSCT) 
      related outcomes of patients with severe combined immunodeficiency (SCID). 
      METHODS: We retrospectively collected data from SCID patients who were diagnosed, 
      followed up and survived at least 2 years after HSCT. RESULTS: Forty four SCID 
      patients were included in the study. Median age of HSCT and follow-up period 
      after HSCT were 7.1 months and 8.7 years, respectively. Human leukocyte antigen 
      (HLA) identical donors were used in 77.3% (n = 34) of the patients (23 siblings, 
      six fathers, two mothers, three extended family donors), HLA 1-2 mismatched 
      family donors in 11.3% (n = 5), and haploidentical family donors in 11.3% 
      (n = 5). CD3 and CD19 counts were normal in more than 90% and in 45.4% at last 
      follow-up, respectively. Intravenous immunoglobulin (IVIG) could be stopped in 
      72.7% (n = 32) after HSCT. B+ SCID patients had better CD19 counts than B- (p 
      < .001). T cell numbers, lymphocyte proliferation, IVIG need, immunoglobulin 
      levels, antibody responses did not differ among B- and B+ immunophenotypes. Acute 
      graft-versus-host disease (GVHD) was less in bone marrow transplanted patients 
      (19.4%) than peripheral stem cell (58.3%) transplanted ones (p = .024). There was 
      no correlation between age at transplantation and immune reconstitution. At the 
      last follow-up, 70.2% and 78.3% of the patients had body weight and height above 
      3(rd) percentile, respectively. CONCLUSION: The immune reconstitution and the 
      growth were normal in the majority of SCID patients after HSCT. It may be 
      rational to use bone marrow instead of peripheral stem cell, as acute GVHD was 
      less in bone marrow transplanted patients.
FAU - Demirtas, Duygu
AU  - Demirtas D
AUID- ORCID: 0000-0002-3662-4355
AD  - Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, 
      Turkey.
FAU - Cagdas, Deniz
AU  - Cagdas D
AUID- ORCID: 0000-0003-2213-4627
AD  - Department of Pediatrics, Division of Immunology, Hacettepe University Faculty of 
      Medicine, Ankara, Turkey.
FAU - Turul Ozgur, Tuba
AU  - Turul Ozgur T
AUID- ORCID: 0000-0003-4545-6151
AD  - Department of Pediatrics, Division of Immunology, Hacettepe University Faculty of 
      Medicine, Ankara, Turkey.
FAU - Kuskonmaz, Baris
AU  - Kuskonmaz B
AUID- ORCID: 0000-0002-1207-4205
AD  - Department of Pediatrics, Division of Hematology, Hacettepe University Faculty of 
      Medicine, Ankara, Turkey.
FAU - Uckan Cetinkaya, Duygu
AU  - Uckan Cetinkaya D
AUID- ORCID: 0000-0003-3593-6493
AD  - Department of Pediatrics, Division of Hematology, Hacettepe University Faculty of 
      Medicine, Ankara, Turkey.
FAU - Sanal, Ozden
AU  - Sanal O
AUID- ORCID: 0000-0003-3463-7555
AD  - Department of Pediatrics, Division of Immunology, Hacettepe University Faculty of 
      Medicine, Ankara, Turkey.
FAU - Tezcan, Ilhan
AU  - Tezcan I
AUID- ORCID: 0000-0001-7257-4554
AD  - Department of Pediatrics, Division of Immunology, Hacettepe University Faculty of 
      Medicine, Ankara, Turkey.
LA  - eng
PT  - Journal Article
DEP - 20210121
PL  - England
TA  - Immunol Invest
JT  - Immunological investigations
JID - 8504629
RN  - 0 (Immunoglobulins, Intravenous)
SB  - IM
MH  - Follow-Up Studies
MH  - *Graft vs Host Disease/etiology
MH  - *Hematopoietic Stem Cell Transplantation/adverse effects
MH  - Humans
MH  - Immunoglobulins, Intravenous
MH  - Retrospective Studies
MH  - *Severe Combined Immunodeficiency/etiology/therapy
OTO - NOTNLM
OT  - Severe combined immunodeficiency
OT  - hematopoietic stem cell transplantation
OT  - immune reconstitution
EDAT- 2021/01/22 06:00
MHDA- 2022/06/03 06:00
CRDT- 2021/01/21 05:23
PHST- 2021/01/22 06:00 [pubmed]
PHST- 2022/06/03 06:00 [medline]
PHST- 2021/01/21 05:23 [entrez]
AID - 10.1080/08820139.2020.1869776 [doi]
PST - ppublish
SO  - Immunol Invest. 2022 May;51(4):739-747. doi: 10.1080/08820139.2020.1869776. Epub 
      2021 Jan 21.