7. First CD3/CD45RA depleted haploidentical transplant for severe combined immunodeficiency disease with Rotavirus gastroenteritis in Vietnam
Main Article Content
Abstract
Severe combined immunodeficiency includes inherited diseases with impairment of T cells development, often associated with profound defects of B and/or NK cells differentiation. Rotavirus infection is responsible for severe acute gastroenteritis and over 400,000 deaths a year in children aged < 5 years, especially in low and middle-income countries. We report a nine-month-old girl presented with failure to thrive left axillary lymphadenitis post-BCG-vaccination, persistent, nonvaccine-associated rotavirus gastroenteritis. An immune evaluation revealed absent T lymphocytes, B lymphocytes, normal NK cells with genetic testing confirmed RAG2 severe combined immunodeficiency. Rotavirus gastroenteritis persisted despite the administration of oral immunoglobulin pre-transplantation. The patient was treated by a haploidentical transplant -from mother as a donor using CD3/CD45RA+ depletion kit, CliniMACs system (Miltenyi). The first transplant was performed without conditioning. One month post-transplant, rotavirus was cleared. The second transplant was performed three months later, with myeloablative conditioning (Bu-Flu-rATG). Neutrophils engrafted on day +10 post-transplant, platelet engrafted on day +45. Whole blood chimerism on day +28 post 2nd transplant was 100%. Serum IgG level has been normalized without IVIG infusion three months post 2nd transplant.
Article Details
Keywords
SCID, Rotavirus gastroenteritis, haplo transplant, CD3/CD45RA depletion
References
2. Lipstein EA, Vorono S, Browning MF et al. Systematic evidence review of newborn screening and treatment of severe combined immunodeficiency. Pediatrics. 2010; 25: e1226-35.
3. Kobrynski LJ, Mayer L. Diagnosis and treatment of primary immunodeficiency disease in patients with gastrointestinal symptoms. Clin Immunol. 2011; 139:238-248.
4. Saulsbury FT, Winkelstein JA, Yolken RH. Chronic rotavirus infection in immunodeficiency. J Pediatr. 1980; 97: 61-5.
5. Patel NC, Hertel PM, Estes MK et al. Vaccine-acquired rotavirus in infants with severe combined immunodeficiency. N Engl J Med. 2010; 362(4): 314-319.
6. GBD Diarrhoeal Diseases Collaborators. Estimates of global, regional, and national morbidity, mortality, and aetiologies of diarrhoeal diseases: a systematic analysis for the Global Burden of Disease Study 2015. Lancet Infect Dis. 17 (2017), pp. 909-948.
7. Dvorak CC, Cowan MJ, Logan BR et al. The natural history of children with severe combined immunodeficiency: baseline features of the first fifty patients of the primary immune deficiency treatment consortium prospective study 6901. J Clin Immunol. 2013; 33(7): 1156- 64.
8. Pai SY, Logan BR, Griffith LM et al. Transplantation outcomes for severe combined immunodeficiency, 2000-2009. N Engl J Med. 2014; 371(5): 434- 46.
9. Touzot F, Neven B, Dal-Cortivo L et al. CD45RA depletion in HLA-mismatched allogeneic hematopoietic stem cell transplantation for primary combined immunodeficiency: A preliminary study. J Allergy Clin Immunol. 2015; 135(5): 1303-9 e1-3.
10. Alexander E, Hommeida S, Stephens MC et al. The Role of Oral Administration of Immunoglobulin in Managing Diarrheal Illness in Immunocompromised Children. Paediatr Drugs. 2020 Jun; 22(3): 331-334.
11. Yoshihara S, Tateishi U, Ando T et al. Lower incidence of Bronchiolitis obliterans in allogeneic hematopoietic stem cell transplantation with reduced-intensity conditioning compared with myeloablative conditioning. Bone Marrow Transplant. 2005 Jun; 35(12): 1195-200.
12. Filipovich AH, Weisdorf D, Pavletic S et al. National Institutes of Health consensus development project on criteria for clinical trials in chronic graft-versus-host disease: I. Diagnosis and staging working group report. Biol Blood Marrow Transplant. 2005; 11: 945-956.
13. Kim SW, Rhee CK, Kim YJ et al. Therapeutic effect of budesonide/formoterol, montelukast and N-acetylcysteine for bronchiolitis obliterans syndrome after hematopoietic stem cell transplantation. Respir Res. 2016; 17(1): 63.