[CIS PIDD] Posting for Listserve

[Sullivan, Kathleen]

GATA2 deficiency perhaps?
Look at monocyte number and NK cell number.

Kate Sullivan, MD PhD
Wallace Chair 
Chief of Allergy Immunology
ARC 1216 CHOP
3615 Civic Center Blvd.
Philadelphia, PA 19104
(p) 215-590-1697
(f) 267-426-0363


> On Mar 17, 2015, at 2:12 PM, Michael Clayton <mhclayton at msn.com> wrote:
> 
> 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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