Abstract
Severe Combined Immunodeficiency (SCID) is one of the most severe forms of primary immunodeficiency (PID). Complications of BCG vaccination, especially disseminated infection and its most severe forms, are known to occur in immunodeficient patients, particularly in SCID. A carefully taken family history before BCG injection as well as delaying vaccination if PID is suspected could be a simple and effective method to avoid inappropriate vaccination of an immunodeficient child in some cases until the prospect of newborn screening for SCID has been fully developed. We describe a patient with a very early diagnosis of SCID, which was suspected on the basis of the previous death of two siblings younger than one year due to severe complications secondary to the BCG vaccine. We suggest that a family history of severe or fatal reactions to BCG should be included as a warning sign for an early diagnosis of SCID.
Highlights
Severe combined immunodeficiency (SCID) represents a group of heterogeneous diseases that predominantly impair the development of T cells
We report a patient with a very early diagnosis of SCID, which was suspected on the basis of the previous death of two siblings younger than one year due to
As we have shown in this article, a family history of severe complications in response to the BCG vaccine can be an important tool for the early diagnosis of SCID in infants
Summary
Severe combined immunodeficiency (SCID) represents a group of heterogeneous diseases that predominantly impair the development of T cells. The infant received the BCG vaccine at seven days of age and progressed without abnormalities until the third month, when he was hospitalized due to important weight loss, oral candidiasis and severe pneumonia, with no improvement with antibiotic therapy. The infant was transferred to the Intensive Care Unit due to important clinical worsening and died at five months of age. Serial blood counts showed anemia and lymphopenia (always below 800 cells/mm3) and blood culture revealed Mb growth. The BCG vaccine was not applied and the child was referred to the Primary Immunodeficiency Outpatient Clinic for investigation. At seven days of life, laboratory tests were performed: a blood count revealed lymphopenia, quantitation of lymphocyte subtypes by flow cytometry revealed an important reduction of CD4+ and CD8+ cells (Table 1); a radiography and computed tomography revealed the absence of the thymus. The child is four years old, has no infections and shows appropriate neuropsychomotor and weight-height development
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