Question

The case of Basil Ransom Basil Ransom was 6 years old when he was referred to the Children's Hospital because of a history of recurrent ear infections and two pneumonias. Even after placement of ear tubes to ventilate the middle ear and prevent the accumulation of fluid and infections, he continued to have monthly infections. On physical examination, he was noted to have a wobbly gait and two dilated superficial blood vessels on his conjunctivae and the outer part of his ear. The pediatrician tested Basil's serum immunoglobulin and found total IgG to be at 219 mg/dl (normal 600-1500 mg/dl) with no detectable IgG2 and IgG4, no detectable IgA, and an elevated level of IgM of 205 mg/dl (normal 22-100 mg/dl). Basil has a positive antibody titer to only 1 of 14 pneumococcal (Streptococcus pneumoniae) subtypes tested, and no detectable antibodies against Haemophilus influenzae, although he had received both the pneumococcal and H. influenzae vaccines. His antibody titer to tetanus was normal. 1. What does this suggest? At the Children's Hospital, a FACS analysis of Basil's peripheral blood mononuclear cells (PBMCs) was notable for low numbers of CD4+ cells, CD8+ cells, and B cells. Proliferation of Basil's PBMCs in response to phytohemagglutinin was reduced, but not absent. Because of the history of recurrent infections, ataxia-telangiectasias, hypogammaglobulinemia, and lymphopenia, the diagnosis is confirmed. 2. What would be the next laboratory test?

          The case of Basil Ransom

Basil Ransom was 6 years old when he was referred to the Children's Hospital because of a history of recurrent ear infections and two pneumonias. Even after placement of ear tubes to ventilate the middle ear and prevent the accumulation of fluid and infections, he continued to have monthly infections. On physical examination, he was noted to have a wobbly gait and two dilated superficial blood vessels on his conjunctivae and the outer part of his ear.

The pediatrician tested Basil's serum immunoglobulin and found total IgG to be at 219 mg/dl (normal 600-1500 mg/dl) with no detectable IgG2 and IgG4, no detectable IgA, and an elevated level of IgM of 205 mg/dl (normal 22-100 mg/dl). Basil has a positive antibody titer to only 1 of 14 pneumococcal (Streptococcus pneumoniae) subtypes tested, and no detectable antibodies against Haemophilus influenzae, although he had received both the pneumococcal and H. influenzae vaccines. His antibody titer to tetanus was normal.

1. What does this suggest?

At the Children's Hospital, a FACS analysis of Basil's peripheral blood mononuclear cells (PBMCs) was notable for low numbers of CD4+ cells, CD8+ cells, and B cells. Proliferation of Basil's PBMCs in response to phytohemagglutinin was reduced, but not absent. Because of the history of recurrent infections, ataxia-telangiectasias, hypogammaglobulinemia, and lymphopenia, the diagnosis is confirmed.

2. What would be the next laboratory test?

Added by Laura F.

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