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

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...

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Bibliographic Details
Published in:Indian journal of pediatrics 2019-07, Vol.86 (7), p.584-589
Main Authors: Aluri, Jahnavi, Gupta, Maya R., Dalvi, Aparna, Mhatre, Snehal, Kulkarni, Manasi, Desai, Mukesh, Shah, Nitin K., Madkaikar, Manisha R.
Format: Article
Language:English
Subjects:
Female
Gynecology
Humans
Immunity, Humoral
Infant
Lymphocyte Count
Lymphopenia - complications
Lymphopenia - immunology
Male
Medicine
Medicine & Public Health
Original Article
Pediatrics
Severe Combined Immunodeficiency - complications
Severe Combined Immunodeficiency - immunology
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Summary: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.
ISSN:0019-5456
0973-7693
DOI:10.1007/s12098-019-02904-9