[CIS PIDD] [cis-pidd] VS: Patient question with low T & B cells

[Seppänen Mikko]

Dear Lynn

Remember to check her monocyte count. If low/suspiciously lowish, check pDC and mDC in peripheral blood, if low seq GATA2 (if neg, check for GATA2 haploinsufficiency).

I would also check anti-PnP responses (if missing?) and B immunophenotyping, could be besides "thymoma in the making" also  "CVID in the making". When chest X-ray, do high resolution as well to exclude bronchiectasis (and signs of PAP).

CD45RA/RO and Tgamma/delta counts would help to assess, if combined ID.

Specifically CD8's are low in CD3gamma def (autoimmunity? thyroid autoimmunity?, ID can be very mild) and in different MHCI defs (for the latter evidently no skin granulomas, sepsis, bronchiectasis+severe emphysema?).

Mikko

Mikko Seppänen MD, Helsinki, Finland

________________________________
Lähettäjä: Wiens, Lynn A MD [mailto:lawiens at saintfrancis.com]
Lähetetty: 4. huhtikuuta 2014 20:23
Vastaanottaja: CIS-PIDD
Aihe: [cis-pidd] Patient question with low T & B cells


I have a 52 y/o white female who came to the office with oral thrush and persistent coughing. Her PFTs are unremarkable and her oral thrush resolved with Diflucan.  As part of her evaluation, I obtained a lymphocyte panel which reveals the following (I did repeat the test to confirm)

1.       CD4+/CD3+  46 (nl 410-1590)

2.      CD8+/CD3+ 1678 (nl 190-1120)

3.      CD4+/CD8+ 0.03 (0.90-3.5)

4.      CD19 B lymphs 20 (200-1300)

She has surprisingly good antibody levels given her low CD19 cts.  My question is:

1.      Other than oral thrush, she doesn't have serious infections yet.  Is there any additional evaluation you would recommend?

2.      Any other treatment at this time in addition to supportive care?  I am quite concerned with that low CD4+ and CD 19 counts...how do I prevent the "time bomb?"

Thanks to all!

Lynn A. Wiens, MD

Warren Clinic

6160 South Yale Avenue  Tulsa, OK 74136

Specializing in the care of Allergy, Asthma, Sinus disease, and Chronic Infections

http://tulsaallergynews.com

Office: 918-495-2636

Cell: 918-550-1200

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