[CIS PIDD] Posting for Listserve

[Carla Gianelli]

Hello,

I agree with all the differential diagnosis discussed ; I would  first rule
out secondary causes: malignancy, autoimmune disease, gastrointestinal
disease, and immunosuppressive.
Posible treatment for your patient: Pentaglobin ® (IVGG enrich with IgM)
Best,

Carla Gianelli.

Servicio de Inmunología.
Hospital Ramon y Cajal
Madrid. Spain.

2015-03-17 19:12 GMT+01:00 Michael Clayton <mhclayton at msn.com>:

> I have had trouble posting over the past week, and would like to try again:
>
> Dear Colleagues:
>
> I would appreciate your thoughts on a 28 year old woman referred to me for
> evaluation by her gynecologist after presenting with scattered areas of
> high-grade squamous vulvar intraepithelial lesions, (VIN 3),  with several
> other areas of VIN 1 level lesions- all highly associated with HPV.  She
> also had perineal condylomata, and numerous flat warts have appeared on her
> face over the past few years.  She recalls:  a diagnosis of bronchiectasis
> as a teen (last chest CT 2002)-no pulmonary follow up in years, recurrent
> respiratory infections when young, having low white blood counts, and a
> normal response to immunizations.  She has a distant history of thoracotomy
> and thyroid surgery at 18 months- at this point, I don’t yet know why.
> Reactive airways disease during URI’s, but she does not use a daily
> controller.
> No familial history for recurrent infections or known immune disease.
>
> Labs from GYN:
> IgM <6, IgG and IgA said to be “normal"
> HIV negative
> Absolute CD4 count 342
>
> I ordered additional labs:
> Normal WBC and other parameters on CBC w/ diff
> IgG 1130, IgA 101, IgM<6, IgE 2.0
> CH50=110
> Flow Cytometry:  %CD4 =26 (32-64), absolute CD4=434, CD4/CD8=0.57
> (0.80-3.90), Absolute CD45RA =50 (130-1100)— all other parameters were WNL.
> Pneumococcal IgG titers= 0/14 “protective range", tetanus=0.1 IU/ml
> Mitogen response= absent to candida, “low normal to tetanus, PHA and ConA,
> nl. response to PWM”
>
> I then immunized with PV23 and tetanus.  Six weeks later, post
> immunization titers to pneumococcus= 0/14 > 1.0 ug/mL, tetanus=2.6.
>
> I am attempting to get her past medical history and labs together as well
> as update her pulmonary condition, but so far she appears to have a
> selective IgM deficiency, which I have never seen before.  I understand
> that this can be associated with malignancy and autoimmunity, and plan to
> screen with at least an autoimmune panel.  Given the severity of her
> medical problems to date and lack of response to the carbohydrate-based
> antigen, I was planning on starting IVIG knowing that while this would not
> replace IgM, it might help with what is presumably a dysregulated immune
> system.  Any other thoughts/suggestions regarding further workup or
> treatment appreciated.
>
> Thanks to everyone,
> Michael H. Clayton, MD, MPH
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