[CIS PIDD] [cis-pidd] CVID in late pregnancy

[CIS-PIDD]

Hi Richard,

Clearly your patient very likely has some form of PID.
Thus to be on the safe side I personally would give IgG temporarily - for the safety of both mother and newborn. After pregnancy, I would stop it (vaccine responses) and look for the specific diagnosis.

I would not yet call her disease CVID nor IgG deficiency. Ig levels during normal pregnancies at different timepoints have been studied, among other lab parameters (by Swedes, some years ago), and her IgG, IgA and IgM levels seem median to me. Like hemoglobin, they are physiologically diluted. I try to find that ref and send it to all, if wanted?

I would try to rule out NEMO and ALPSs among others from the mother. The exact dg may be vital to know even for the child.

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Was the first newborn clearly thrombopenic as well? Was CGH performed? Deletional sdr like severe 11qter or DGS?

Kind regards,

Mikko

Oyl Mikko Seppänen
Harvinaissairauksien yksikkö (HAKE)

Chief, Rare Disease Center,
Helsinki University Hospital (HUH)
FINLAND

phone +358 947180201
GSM +358 50 4279606
fax +358 9 47174703

CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> kirjoitti 15.10.2015 kello 2.43:

Yes-  no harm done anyway.  We have had 2 babies with sepsis in the first months of life, born to nearly agamma moms who were not yet on ivig. (One died)   I assume lack of maternal Ig was the cause.

Sent from my iPhone

On Oct 14, 2015, at 5:14 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> wrote:

I saw a 28 year old woman yesterday to counsel about SCID. Her first baby and multiple congenital anomalies, zero TRECs, profound lymphopenia and died at a few weeks of age of heart disease and post-op infection. GeneDx panel of 18 SCID genes was negative. No specific diagnosis.

Current patient is due to deliver in three weeks. Her history includes:
septic arthritis, N. meningitidis meningitis after vaccination, five episodes of pneumonia including an MRSA and a Klebsiella, multiple episodes of bronchitis and three episodes of adenopathy sufficient to merit biopsy - all reactive lymphoid hyperplasia. She has had some evaluation in the past that is not yet available. She has been said to be vaccine non-responsive. Her screening studies are:

Immunoglobulin G, Qn, Serum [L]  526 mg/dL   700-1600
Immunoglobulin A, Qn, Serum 129 mg/dL     91-414
Immunoglobulin M, Qn, Serum  [H]  256 mg/dL   40-230

Would you treat her with immunoglobulin prior to delivery?

Thanks,
Richard

--
Richard L. Wasserman, MD, PhD
Allergy Partners of North Texas
7777 Forest Lane, Suite B-332
Dallas, Texas 75230
Office (972) 566-7788
Fax (972) 566-8837
Cell (214) 697-7211

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