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

[Nacho Gonzalez]

Dear Dr. Church,

the last 2 XLA patients I have seen recently were under the first year of
life. They had poor vascular access... every IVIG infusion was a pain. Now
they are under SCIG and doing fine. But going back to your patient, I do
not think she needs IgG replacement therapy if she is free of infections.

Best regards,

Luis Ignacio Gonzalez-Granado
Immunodeficiencies Unit
Pediatric Hematology & Oncology Unit
Hospital 12 octubre
Madrid. Spain

2015-07-22 20:19 GMT+02:00 Verbsky, James <jverbsky at mcw.edu>:

>  Not sure if this will work as well in teliangiectasia but in other PLE
> states subQ has been beneficial…might be tough in an 18mo..but it’s a
> thought
>
>
>
> james
>
>
>
> *From:* Church, Joseph [mailto:JChurch at chla.usc.edu]
> *Sent:* Wednesday, July 22, 2015 1:08 PM
> *To:* CIS-PIDD
> *Subject:* [cis-pidd] Baby with intestingal lymphangiectasia
>
>
>
> 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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