[CIS PIDD] [cis-pidd] Recurrent infection, no IgA, high IgM, normal IgG

Mon Jul 7 13:56:21 EDT 2014

I have two patients like this one who have benefited from IgG therapy. The
way to get pneumococcal antibody titers done from Canada is to complete the
foreign laboratory test authorization form and send the test to a reliable
lab in the US, there are several.
Richard Wasserman
Dallas

On Mon, Jul 7, 2014 at 9:42 AM, Cunningham-Rundles, Charlotte <
charlotte.cunningham-rundles at mssm.edu> wrote:

>  Just a note:
>
>  Alper reported in  the JEM ( J Exp Med.
> <http://eresources.library.mssm.edu:2132/pubmed/1531063#> 1992 Feb
> 1;175(2):495-502)   that persons homozygous for HLA 1/8/DR3  were likely to
> be non responders to hepatitis B vaccine.  This HLA type is linked to  IgA
> deficiency.    If he has this typing he likely to be  a genetic non
> responder and I would not use Ig for him on this basis.
>
>  Not sure I understand the other titers you cite here ( what is positive
> and what is not )  ; how about antibodies to pneumococcal vaccine? Some IgA
> deficient persons ( with normal IgG2)  do not produce IgG antibodies to
> various vaccines, as reported  previously.
>
>
>
>  Charlotte
>
>
>
>  Charlotte Cunningham-Rundles MD PhD
> David S Gottesman Professor
> Department of Medicine
> 1425 Madison Avenue
> New York City  NY 10029
> Charlotte.Cunningham-Rundles at mssm.edu
>
>
>  On Jul 7, 2014, at 7:47 AM, Michelle Halbrich <
> michelle.halbrich at hotmail.com> wrote:
>
>  Hi everyone,
>
> I was wondering if I could ask your thoughts on a patient I saw. I would
> appreciate additional suggestions:
>
> 27 year old male with a history of IgA deficiency, presented prior to
> dental surgery for the “go-ahead” from Immunology for the surgery.
>
> History is significant for “18” T-tube surgeries for recurrent OM, 4
> episodes of pneumonia, 2 sinus infections, gastroenteritis around once a
> year (when everyone else is sick), a fungal skin infection 2 years ago
> treated with creams, a dental abscess (reason for the dental surgery), 2-3
> UTIs.
>
> He has a history of “asthma” diagnosed at the age of 6, difficult to
> treat, on multiple puffers. He has symptoms of AR.
>
> He was vaccinated for Hep B, but required a booster because “it did not
> take”.
>
> Family history is significant for the maternal grandfather who had 4
> malignancies, including vocal cancer.
>
> He is a singer.
>
> I had ordered vaccine serology as part of the initial workup, and when he
> was not protected against MMR, his family MD vaccinated him (he did fine
> after receiving the live vaccine).
>
> In January 2014, he was in an MVA. He was started on prophylactic
> antibiotics while awaiting workup (and referral to the immunology centre).
> May 2014 he was admitted for a few days with pneumonia.
>
> Labs: this is what I have:
>
> - Low IgA, high-ish normal IgG, and elevated IgM on multiple samples: *IgA
> < 0.1*, IgG 14.9, *IgM 5.4*
>
> - WBC 3.8, low neutrophils of 1.39, otherwise unremarkable CBC; normal
> lymphocyte phenotyping with CD19 count of 140, CD8 6178, NK 110;
> isohaemagluttinin anti-A titre 1:8, anti-B titre 1:128; normal PHA
> lymphocyte stimulation assay of 614.
>
> - normal total protein and albumin
>
> - Diphtheria serology 0.34,0.29 IU/mL, Tetanus serology 0.22,0.17 IU/mL
> (done twice).
>
> - Non-reactive Hep B (0.54mIU/mL) despite vaccination; non-reactive
> measles (129.54 mIU/L), mumps (15.03 RU/mL), intermediate rubella (5.1
> IU/mL), that responded to vaccination (measles 910.95 mIU/mL, mumps 92.69
> RU/mL, rubella 7.1 IU/mL).
>
> - SPEP shows diffuse polyclonal increase in gamma globulins, increased
> alpha 1 and alpha 2 (consistent with acute phase reaction), NOT suggestive
> of a monoclonal pattern.
>
> - HIV antigen and antibody negative.
>
> - CT chest, Jan 2014: diffuse bronchial wall thickening, no bronchial
> dilation, numerous peribronchovascular ill-defined nodular opacities and
> branching opacities likely related to inflammatory or infectious processes.
>
> - Abdominal Ultrasound: normal
>
> - Spine MRI, May 2014: Schmorl nodes involving T11-T12, T12-L1, L1-L2,
> mild disc desiccation at L1-L2 and L2-L3, minimal dorsal disc bulge at
> L4-L5, L5-S1, with no evidence of disc herniation
>
> Any suggestions? I suspect he needs Ig replacement, despite the response
> to the MMR vaccine. What about CD40? CD40L? I have referred him to
> Respirology and the appointment is pending.
>
> Thank you!
>
>
> Michelle Halbrich, MD, FRCPC
> Paediatrician, Clinical Immunology and Allergy
> Toronto, Canada
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>
> Charlotte Cunningham-Rundles MD PhD
> David S Gottesman Professor
> Department of Medicine
> 1425 Madison Avenue
> New York City  NY 10029
> Charlotte.Cunningham-Rundles at mssm.edu
>
>
>
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-- 
Richard L. Wasserman, MD, PhD
DallasAllergyImmunology
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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