[CIS PIDD] [cis-pidd] 62yo M with chronic infections, slightly abnormal labs

[Yeşim Yılmaz Demirdağ]

Hi Shayna,
has CF been ruled out?
Yesim

Yesim Yilmaz Demirdag, MD
Columbia University Medical Center
3959 Broadway Suite 107N
New York, NY 10032
Phone: (212) 305 2300



On Thu, Jul 10, 2014 at 1:39 PM, Riedl, Marc <mriedl at ucsd.edu> wrote:

>  Curious what PFTs/DLCO and Chest CT look like.
>
>  Marc Riedl, MD, MS
> Associate Professor of Medicine
> Division of Rheumatology, Allergy & Immunology
> University of California, San Diego
>
> 9500 Gilman Dr, Mail Code 0732
>
> La Jolla, CA  92093
> Tel 858.822.6766  Fax 858.642.3791
>
>
>
> From: Shayna Burke <shaynaburke at yahoo.com>
> Reply-To: CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
> Date: Thursday, July 10, 2014 6:49 AM
> To: CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
> Subject: Re:[cis-pidd] 62yo M with chronic infections, slightly abnormal
> labs
>
>
> I recently saw this patient in my private practice office and was looking
> for any advice on an actual diagnosis, any further testing that should be
> done, and treatment suggestions.  Thanks in advance.
>
> He is a 62 year old gentleman who was diagnosed with asthma in his 30s
> (1983) after taking Inderal (propranolol) for an ocular migraine and within
> a few days of starting this medications suddenly developed severe asthma.
> He has been hospitalized 2x for asthma, with 1x in the ICU.  He started
> prednisone in 1987 after he got a flu shot and then subsequently developed
> a severe asthma exacerbation, and has never been able to get off of the
> prednisone.  He used to require albuterol 4x/day, but now is much better
> controlled.  He is on numerous medications for his asthma, including
> prednisone 10mg daily, theophylline 300mg BID, Singulair 10mg daily, Dulera
> 200/5 mcg 2 puffs BID, Alvesco 160mcg 2 puffs BID, and Xolair 300mg SQ
> Q4weeks.
>
> He also has chronic otitis and sinus problems, including s/p bilateral
> mastoidectomies (right 1998, left 2010) and numerous myringotomy tubes, but
> with continued frequent otitis.  At the time that I saw him, he actually
> had new onset of green pus draining from his previously healed incision of
> his left mastoidectomy, which ended up growing pseudomonas.  In the past
> cultures of his ear have grown MRSA, for which he was treated with Bactrim
> x2months.     He has had pneumonia 2 times in the past (confirmed by
> CXR), 1 of which he was hospitalized for.  He also has a history of nasal
> polyps, s/p sinus/polyp surgery 3x (last in 1994).  He has not had any
> regrowth of the nasal polyps since remaining on fluticasone nasal spray
> after his last sinus surgery.
>
>
> *Labs*:
>
>
>    - normal CBC with dif aside from slightly high WBC at 11.9, slightly
>    high neutrophil % at 88.4% and total neutrophil count at 10.5 k/UL, and low
>    lymphocyte percent at 5.8% and absolute at 0.7,
>    - normal IgA at 105,  slightly low total IgM at 41 (nl 45-281),
>    elevated total IgE at 417 (on Xolair)
>    - normal total IgG at 900, IgG1 at 438, IgG2 at 328, IgG3 at 41, and
>    elevated IgG4 at 196,
>    - normal alpha-1-antitrypsin level at 141,
>    - protective diphtheria Ab at 0.46, protective tetanus Ab at 1.95
>    - “pre vaccination” (est last Pneumovax 5-10 years ago):  only 11 out
>    of 23 protective,
>    - “post vaccination” with Pneummovax:  protective only to 14 out of 23
>    Pneumococcal serotypes with only 3-4 that had a 4fold increase and 7
>    serotypes actually went down from pre to post vaccination titers.
>    - normal complement level and normal C4 level,
>    - low total lymphocyte count (CD45) at 0.8 (nl 0.82-2.84) with normal
>    Tcell and NK cell %s but low Bcell % at 3%, low total CD3 Tcell number at
>    529 (nl 550-2202), low Bcell number at 17 (nl 45-409), and low CD4 helper
>    cells at 336 (nl 365-1437).
>    - Of note, there were 14% CD3+CD4+CD8+ (double positive) Tcells
>    present.
>
>
> Given his chronic infections, despite his normal total IgG, I was planning
> on starting a trial of SCIG replacement.
>
> I also recently ordered an SPEP and HIV screen, which are pending.  He is
> having a ciliary biopsy by ENT this week as well.
>
> Any suggestions would be greatly appreciated.   Thanks in advance.
>
>
>
> Sincerely,
>
>
>
> Shayna Burke, MD
>
> Allergy and Asthma Family Care
>
> 928 Farmington Ave
>
> West Hartford, CT 60107
>
> Phone:  860-233-6293
>
>
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