[CIS PIDD] [cis-pidd] Baby with intestingal lymphangiectasia

[Church, Joseph]

Colleagues:

I have consulted on an 18 month-old girl with intestinal lymphangiectasia and unilateral lymphedema.  She has experienced no infectious complications.

However, labs demonstrate
            Serum albumin 2.3 g/dL
            IgG      83 mg/dL
            IgA      <7 mg/dL
            IgM      27 mg/dL
            Tetanus antibody 0.49 IU/mL (considered protective)
            Haemophilus influenzae antibody 0.48 mcg/mL (considered unprotective)
            Pneumococcal antibody response to Prevnar 13 positive (>1.3 mcg/ml) for 10 of 12 serotypes tested.
            CD3+ 46% (1399/mcL)
            CD4+ 14% (328/mcL)
            CD8+ 46% (1113/mcL)
            CD19+ 24% (590/mcL)
            NK 16% (391/mcL)
            PHA and PWM responses decreased (~ 25% of concurrent control)

The child is clearly losing protein, immunoglobulins and T-cells (CD4+ > CD8+), but has fair circulating antibody responses to tetanus toxoid and conjugated pneumococcal vaccine.

I would appreciate your opinions regarding immunoglobulin replacement therapy.

Joe Church, MD
Children's Hospital Los Angeles




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