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Lymphopenia and Severe Combined Immunodeficiency (SCID) - Think Before You Ink

  • Jahnavi Aluri
  • Maya R. Gupta
  • Aparna Dalvi
  • Snehal Mhatre
  • Manasi Kulkarni
  • Mukesh Desai
  • Nitin K. Shah
  • Manisha R. MadkaikarEmail author
Original Article

Abstract

Objectives

Severe combined immunodeficiency (SCID) represents one of the most severe forms of Primary immunodeficiency (PID) disorders, characterized by T cell lymphopenia (TCL) and lack of cellular and humoral immune responses. However, not all patients with low T cell lymphocyte counts may have an abnormal T cell immunity and the observed TCL may be a temporary suppression resulting from transient lymphopenia secondary to severe infections. In such cases, it is necessary to estimate the severity of the observed TCL by assessing thymic capabilities.

Methods

In this study, patients clinically suspected of SCID were evaluated for lymphocyte subsets analysis, naïve T cells and T cell receptor excision circles (TREC).

Results

Patients with transient lymphopenia had detectable TREC levels and normal naïve T cells subsets. Normalization of absolute lymphocyte counts, and T cells was seen in the patients after a short duration.

Conclusions

The authors highlight the importance of detailed immunological investigations in an infant with severe infections and lymphopenia before labeling the infant as SCID.

Keywords

SCID TREC Naïve T cells Transient lymphopenia 

Notes

Acknowledgments

The authors thank Dr. Daniel Douek, Vaccine Research Center, National Institute of Allergy and Infectious Diseases, USA, for providing the hTREC plasmid for performing the TREC assay.

Authors’ Contributions

JA wrote the manuscript. MRG, AD, SM, MK performed the various laboratory tests. MK, NKS provided clinical care to the patients. MRM supervised the study and will act as guarantor for this paper.

Compliance with Ethical Standards

Conflict of Interest

None.

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Copyright information

© Dr. K C Chaudhuri Foundation 2019

Authors and Affiliations

  • Jahnavi Aluri
    • 1
  • Maya R. Gupta
    • 1
  • Aparna Dalvi
    • 1
  • Snehal Mhatre
    • 1
  • Manasi Kulkarni
    • 1
  • Mukesh Desai
    • 2
  • Nitin K. Shah
    • 3
  • Manisha R. Madkaikar
    • 1
    Email author
  1. 1.Department of Pediatric Immunology and Leukocyte BiologyNational Institute of Immunohematology (ICMR)MumbaiIndia
  2. 2.Division of ImmunologyBai Jerbai Wadia Children’s HospitalMumbaiIndia
  3. 3.Pediatric Hematology-OncologyP. D. Hinduja National Hospital & Research CenterMumbaiIndia

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