[CIS PIDD] Posting for Listserve

[Bodo Grimbacher]

Dear all,
One of our ICOS-deficient patients died of HPV-associated vulvar cancer.
In light of Geha¹s recent publication of a new ICOS-deficient family with
opportunistic PJP this may be a DD also for your patient?
Best, Bodo

****************************************
Univ.-Prof. Dr. med. B. Grimbacher
 
Scientific-Director
CCI-Center for Chronic Immunodeficiency
UNIVERSITÄTSKLINIKUM FREIBURG
Tel.: 0761 270-77731  Fax: -77744
Engesserstraße 4, 79108 Freiburg
bodo.grimbacher at uniklinik-freiburg.de
www.uniklinik-freiburg.de/cci




Am 17/03/15 19:12 schrieb "Michael Clayton" unter <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
>don¹t yet know why.  Reactive airways disease during URI¹s, 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 ³normal"
>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 ³protective range", tetanus=0.1 IU/ml
>Mitogen response= absent to candida, ³low normal to tetanus, PHA and
>ConA, nl. response to PWM²
>
>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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