Article

Beware the lymphopenia: a case of severe combined immunodeficiency.

Department of Allergy and Immunology, The Children's Hospital at Westmead, Sydney, New South Wales, Australia.
Journal of Paediatrics and Child Health (impact factor: 1.28). 08/2011; 47(8):565-7. DOI:10.1111/j.1440-1754.2010.01870.x pp.565-7
Source: PubMed

ABSTRACT We present a case of a 2-month-old boy with partially treated meningitis and suspected Pneumocystis carinii pneumonia. A full blood count revealed profound lymphopenia. The child was diagnosed with adenosine deaminase deficiency, a rare cause of severe combined immunodeficiency (SCID). SCID is an immunological emergency and must be considered in any lymphopaenic infant with opportunistic infection. We discuss adenosine deaminase-deficient SCID, which can involve multiple systems and in which other treatment options apart from bone marrow transplant are available.

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Keywords

2-month-old boy
 
adenosine deaminase deficiency
 
adenosine deaminase-deficient SCID
 
bone marrow transplant
 
immunological emergency
 
lymphopaenic infant
 
opportunistic infection
 
Pneumocystis carinii pneumonia
 
profound lymphopenia
 
rare cause