Recurrent Infections, Diarrhea and Hypereosinophilia

  • Haskologlu Sule
  • Islamoglu Candan
  • Ikinciogullari AydanEmail author


  • Maternal T cell engrafment has been reported in as many as 40% of newborns with severe combined immunodeficiency (SCID)

  • Hypofunctioning T cells may facilitate persistent engraftment of maternal T lymphocytes, leading to alloreactive immune response referred as graft versus host disease (GVHD). Maternal engraftment syndrome typically present in a neonate/infant as rash and/or diarrhea

  • One indicator that maternal T cell engraftment is present, is predominance of either CD4+ or CD8+ T cells since maternally engrafted cells are oligoclonal. A majority of the maternally engrafted T cells have an activated or memory phenotype and express CD45RO

  • HLA typing, PCR-based short tandem repeat (STR) analysis or fluorescence in-situ hybridization (FISH) analysis, can help detect maternal engrafment syndrome


Severe combined immunodeficiency Maternal engraftment syndrome Fluorescence in-situ hybridization CD45RO 


  1. 1.
    Fischer A, Cavazzana-Calvo M, De Saint Basile G, DeVillartay JP, Di Santo JP, Hivroz C, Rieux-Laucat F, Le Deist F. Naturally occurring primary deficiencies of the immune system. Annu Rev Immunol. 1997;15:93–124.CrossRefGoogle Scholar
  2. 2.
    Notarangelo LD. Combined immunodeficiencies with nonfunctional T lymphocytes. Adv Immunol. 2014;121:121–90.CrossRefGoogle Scholar
  3. 3.
    Notarangelo LD. Partial defects of T-cell development associated with poor T-cell function. J Allergy Clin Immunol. 2013;131(5):1297–305.CrossRefGoogle Scholar
  4. 4.
    Villa A, Notarangelo LD, Roifman CM. Omenn syndrome: inflammation in leaky severe combined immunodeficiency. J Allergy Clin Immunol. 2008;122(6):1082–6.CrossRefGoogle Scholar
  5. 5.
    Dvorak CC, Cowan MJ, Logan BR, Notarangelo LD, Griffith LM, Puck JM, Kohn DB, Shearer WT, O’Reilly RJ, Fleisher TA, Pai SY, Hanson IC, Pulsipher MA, Fuleihan R, Filipovich A, Goldman F, Kapoor N, Small T, Smith A, Chan KW, Cuvelier G, Heimall J, Knutsen A, Loechelt B, Moore T, Buckley RH. 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.CrossRefGoogle Scholar
  6. 6.
    Ingle SB, Hinge Ingle CR. Primary intestinal lymphangiectasia: minireview. World J Clin Cases. 2014;2(10):528–33.CrossRefGoogle Scholar
  7. 7.
    Liu C, Duffy B, Bednarski JJ, Calhoun C, Lay L, Rundblad B, Payton JE, Mohanakumar T. Maternal T-cell engraftment interferes with human leukocyte antigen typing in severe combined immunodeficiency. Am J Clin Pathol. 2016;145(2):251–7.CrossRefGoogle Scholar

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© Springer Nature Switzerland AG 2020

Authors and Affiliations

  • Haskologlu Sule
    • 1
  • Islamoglu Candan
    • 1
  • Ikinciogullari Aydan
    • 1
    Email author
  1. 1.Department of Pediatric Allergy and ImmunologyAnkara University School of MedicineAnkaraTurkey

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