[CIS PIDD] [cis-pidd] Low NK function question

[CIS-PIDD]

Hi Jennifer!

About the exact diagnosis, I would contact Jordan Orange and his NK and their genetics lab in Texas...

Prophylactic long term valaciclovir is well tolerated, efficacy against HSV depends on the conscientious and regular use and perfect compliance, thus I use it only in

- highly frequent recurrent proven HSV meningitis and

- recurrent HSV encephalitis (with TLR3 pathway defects) and also in their seronegative carrier relatives

- highly recurrent (> 7-10 recurrences /y for >1 y after primary clinical episode) genital HSV2

(who all usually are extremely compliant and eager to consume 1000mg x2-3/d "forever", though in gHSV2 one usually gets rid of the medication after some years)

ATB

Mikko

Mikko Seppänen MD, PhD
[X]

Head, Rare Disease Center,
Helsinki University Hospital (HUH)
FINLAND

phone +358 947180201
GSM +358 50 4279606
fax +358 9 47174703

CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> kirjoitti 12.10.2016 kello 5.28:

I don’t think that there is any established treatment in this situation. It would be reassuring to know that her CMI function was normal – aside from the phenotype results you provide. A 19 year interval between outbreaks is probably too long for chemoprophylaxis. I wonder, would the shingles vaccine influence her evolution?

Anthony Hayward

From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: Tuesday, October 11, 2016 4:49 PM
To: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Subject: [cis-pidd] Low NK function question

Hi All,

I recently graduated from A/I fellowship and now seeing patients at a private practice. We recently saw a 49 year old Japanese female with recurrent episodes of shingles at the age of 25, 30 and 49 and a history of recurrent mumps-like illness during childhood. No other significant infections or significant PMH.

Quantitative immunoglobulins and lymphocyte subsets/% including NK cells were normal. CBC normal with no monocytopenia. However, NK cell function (via Quest labs) was low at 5 LU30 (decreased activity <7, normal activity 7-125). This was repeated with the same result.

She has undetectable titers to HSV and CMV. Is there anything else that we can offer this patient? Prophylaxis? How should we proceed? Would appreciate any thoughts! Thanks in advance,

--
Jennifer Toh, M.D.
Associate
Adult & Pediatric Allergy, Immunology, Asthma & Sinus Disease
New York Allergy and Asthma, PLLC
261 East 78th St., Floor 4, New York, NY 10075
Tel: 212 517-3300, Fax: 212 517-3303

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