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  1. Article ; Online: Relevance of lymphocyte proliferation to PHA in severe combined immunodeficiency (SCID) and T cell lymphopenia.

    Abraham, Roshini S / Basu, Amrita / Heimall, Jennifer R / Dunn, Elizabeth / Yip, Alison / Kapadia, Malika / Kapoor, Neena / Satter, Lisa Forbes / Buckley, Rebecca / O'Reilly, Richard / Cuvelier, Geoffrey D E / Chandra, Sharat / Bednarski, Jeffrey / Chaudhury, Sonali / Moore, Theodore B / Haines, Hilary / Dávila Saldaña, Blachy J / Chellapandian, Deepakbabu / Rayes, Ahmad /
    Chen, Karin / Caywood, Emi / Chandrakasan, Shanmuganathan / Lugt, Mark Thomas Vander / Ebens, Christen / Teira, Pierre / Shereck, Evan / Miller, Holly / Aquino, Victor / Eissa, Hesham / Yu, Lolie C / Gillio, Alfred / Madden, Lisa / Knutsen, Alan / Shah, Ami J / DeSantes, Kenneth / Barnum, Jessie / Broglie, Larisa / Joshi, Avni Y / Kleiner, Gary / Dara, Jasmeen / Prockop, Susan / Martinez, Caridad / Mousallem, Talal / Oved, Joseph / Burroughs, Lauri / Marsh, Rebecca / Torgerson, Troy R / Leiding, Jennifer W / Pai, Sung Yun / Kohn, Donald B / Pulsipher, Michael A / Griffith, Linda M / Notarangelo, Luigi D / Cowan, Morton J / Puck, Jennifer / Dvorak, Christopher C / Haddad, Elie

    Clinical immunology (Orlando, Fla.)

    2024  Volume 261, Page(s) 109942

    Abstract: Severe combined immunodeficiency (SCID) is characterized by a severe deficiency in T cell numbers. We analyzed data collected (n = 307) for PHA-based T cell proliferation from the PIDTC SCID protocol 6901, using either a radioactive or flow cytometry ... ...

    Abstract Severe combined immunodeficiency (SCID) is characterized by a severe deficiency in T cell numbers. We analyzed data collected (n = 307) for PHA-based T cell proliferation from the PIDTC SCID protocol 6901, using either a radioactive or flow cytometry method. In comparing the two groups, a smaller number of the patients tested by flow cytometry had <10% of the lower limit of normal proliferation as compared to the radioactive method (p = 0.02). Further, in patients with CD3+ T cell counts between 51 and 300 cells/μL, there was a higher proliferative response with the PHA flow assay compared to the
    MeSH term(s) Infant, Newborn ; Humans ; Severe Combined Immunodeficiency/diagnosis ; Lymphopenia/diagnosis ; Neonatal Screening/methods ; T-Lymphocytes ; Cell Proliferation
    Language English
    Publishing date 2024-02-15
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Extramural
    ZDB-ID 1459903-x
    ISSN 1521-7035 ; 1521-6616
    ISSN (online) 1521-7035
    ISSN 1521-6616
    DOI 10.1016/j.clim.2024.109942
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Outcomes following treatment for ADA-deficient severe combined immunodeficiency: a report from the PIDTC.

    Cuvelier, Geoffrey D E / Logan, Brent R / Prockop, Susan E / Buckley, Rebecca H / Kuo, Caroline Y / Griffith, Linda M / Liu, Xuerong / Yip, Alison / Hershfield, Michael S / Ayoub, Paul G / Moore, Theodore B / Dorsey, Morna J / O'Reilly, Richard J / Kapoor, Neena / Pai, Sung-Yun / Kapadia, Malika / Ebens, Christen L / Forbes Satter, Lisa R / Burroughs, Lauri M /
    Petrovic, Aleksandra / Chellapandian, Deepak / Heimall, Jennifer / Shyr, David C / Rayes, Ahmad / Bednarski, Jeffrey J / Chandra, Sharat / Chandrakasan, Shanmuganathan / Gillio, Alfred P / Madden, Lisa / Quigg, Troy C / Caywood, Emi H / Dávila Saldaña, Blachy J / DeSantes, Kenneth / Eissa, Hesham / Goldman, Frederick D / Rozmus, Jacob / Shah, Ami J / Vander Lugt, Mark T / Thakar, Monica S / Parrott, Roberta E / Martinez, Caridad / Leiding, Jennifer W / Torgerson, Troy R / Pulsipher, Michael A / Notarangelo, Luigi D / Cowan, Morton J / Dvorak, Christopher C / Haddad, Elie / Puck, Jennifer M / Kohn, Donald B

    Blood

    2022  Volume 140, Issue 7, Page(s) 685–705

    Abstract: Adenosine deaminase (ADA) deficiency causes ∼13% of cases of severe combined immune deficiency (SCID). Treatments include enzyme replacement therapy (ERT), hematopoietic cell transplant (HCT), and gene therapy (GT). We evaluated 131 patients with ADA- ... ...

    Abstract Adenosine deaminase (ADA) deficiency causes ∼13% of cases of severe combined immune deficiency (SCID). Treatments include enzyme replacement therapy (ERT), hematopoietic cell transplant (HCT), and gene therapy (GT). We evaluated 131 patients with ADA-SCID diagnosed between 1982 and 2017 who were enrolled in the Primary Immune Deficiency Treatment Consortium SCID studies. Baseline clinical, immunologic, genetic characteristics, and treatment outcomes were analyzed. First definitive cellular therapy (FDCT) included 56 receiving HCT without preceding ERT (HCT); 31 HCT preceded by ERT (ERT-HCT); and 33 GT preceded by ERT (ERT-GT). Five-year event-free survival (EFS, alive, no need for further ERT or cellular therapy) was 49.5% (HCT), 73% (ERT-HCT), and 75.3% (ERT-GT; P < .01). Overall survival (OS) at 5 years after FDCT was 72.5% (HCT), 79.6% (ERT-HCT), and 100% (ERT-GT; P = .01). Five-year OS was superior for patients undergoing HCT at <3.5 months of age (91.6% vs 68% if ≥3.5 months, P = .02). Active infection at the time of HCT (regardless of ERT) decreased 5-year EFS (33.1% vs 68.2%, P < .01) and OS (64.7% vs 82.3%, P = .02). Five-year EFS (90.5%) and OS (100%) were best for matched sibling and matched family donors (MSD/MFD). For patients treated after the year 2000 and without active infection at the time of FDCT, no difference in 5-year EFS or OS was found between HCT using a variety of transplant approaches and ERT-GT. This suggests alternative donor HCT may be considered when MSD/MFD HCT and GT are not available, particularly when newborn screening identifies patients with ADA-SCID soon after birth and before the onset of infections. This trial was registered at www.clinicaltrials.gov as #NCT01186913 and #NCT01346150.
    MeSH term(s) Adenosine Deaminase ; Agammaglobulinemia/genetics ; Child, Preschool ; Hematopoietic Stem Cell Transplantation ; Humans ; Infant ; Infant, Newborn ; Severe Combined Immunodeficiency/genetics ; Severe Combined Immunodeficiency/therapy
    Chemical Substances Adenosine Deaminase (EC 3.5.4.4)
    Language English
    Publishing date 2022-06-03
    Publishing country United States
    Document type Clinical Trial ; Journal Article
    ZDB-ID 80069-7
    ISSN 1528-0020 ; 0006-4971
    ISSN (online) 1528-0020
    ISSN 0006-4971
    DOI 10.1182/blood.2022016196
    Database MEDical Literature Analysis and Retrieval System OnLINE

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