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

[Shayna Burke]

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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