[CIS PIDD] [MARKETING][cis-pidd] 18yo M with antibody deficiency

[CIS-PIDD]

Thanks, Mikko.

At this point the patient has been completely asymptomatic for over 5 years.  There is no family history of immune disorders and no physical evidence for splenomegaly or lymphadenopathy.
The family and the primary MD are very good, so I don't think the patient will be lost.

JC

From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: Thursday, September 10, 2015 8:21 AM
To: CIS-PIDD
Subject: Re: [MARKETING][cis-pidd] 18yo M with antibody deficiency

Hi Joe!

Personally, I would continue follow up, but also include a.o.t.:

A) diffusion capacity follow up yearly, more often if sudden dips

B) check naive T cell levels (is it only humoral for sure?) - if not done already?

C) check HRCT at least once - bronchiectasis, other? And if signs suggestive of such, re-do it.

D) yearly check up of IgM, IgG, IgE - will he be losing these as well?

E) spleen size, lymphadenopathy? Family history? If enlarged, maybe even EBV-, CMV-PCR from blood and immunostaining from lymph node biopsy (PIK3CD GOF, -R1 a.s.f.)

And prescriptions for antibiotic courses used early, if prolonged/severe infections.

I would hate, if this person would "fall off the grid"...

T: Mikko Seppänen, Finland

CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> kirjoitti 10.9.2015 kello 16.05:
Colleagues:

I have followed an 18yo male who had a history of recurrent URIs associated with IgA deficiency and poor antibody responses to pneumococcal polysaccharide antigens (Pneumovax).

He has had no significant infections for at least 5 years.

His primary MD wanted to check his immune status prior to the young man going to college.  The patient received Menactra (conjugated N. men A, C, Y, W-135 polysaccharides) and Prevnar (conjugated 13 serotypes of S. pneumo polysaccharides) on July 28.

Labs drawn on September 1 showed:

-       Positive antibodies to N. men A only

-       Positive antibodies to ONE serotype of S. pneumo

-       Normal IgG and Ig M levels.

The lack of response to conjugated polysaccharide vaccines is of concern to me.

Would you suggest:

-       Continue observation and urgent use of antibiotics for febrile episodes?

-       Antibiotic prophylaxis?

-       Immunoglobulin replacement?

-       Other?

-
Thank you for your consideration.

Joe Church
Children's Hospital Los Angeles

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