[CIS PIDD] [cis-pidd] Asymptomatic patient with low ALC, hypogamm, CD4 count 17, and absent mitogen stim - management recommendations?

[CIS-PIDD]

Hi Dr. Schapira,

I would rule out intestinal lymphangectasia with such low lymphocyte counts
and hypogam but without clinical overt infections.

Best wishes,

Boaz

Boaz Palterer, MD
Clinical Immunologist
Department of Clinical and Experimental Medicine
Unit of Allergology and Clinical Immunology
University of Florence, Italy
email: boaz.palterer at unifi.it
cell: +39 392 7169114

On Wed, Nov 22, 2017 at 5:41 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
wrote:

> Dr. Schapira:
>
> I am concerned about the absent mitogen stimulation and the absolute CD4
> count of 17/uL in the context of pulmonary nodules ... out of curiosity,
> what was the positive control value for the T-spot?  I'm not sold on the
> legitimacy of a negative T-spot in a person with 17 CD4 T cells, all named
> Bob.  This is just measuring IFN-gamma production of CD8's / NK's, after
> all.
>
> My second question is how did the infectious disease physician "envision"
> the patient -- did he/she evaluate the patient as a nominally healthy
> patient who might have an infection causing lymphopenia, or an
> immunodeficient patient who might have or be at risk for an infection?  My
> concern is that many tests will run false negatives (e.g.,
> PPD/QuantiFERON/T-spot ... potentially even titers for some pathogens) if
> the patient's T cells don't work to begin with.
>
> I would suggest some screening if it hasn't been done yet ... sputum
> culture X 3 for AFB (to catch TB, NTM's), fungal culture of sputum, and
> depending on his travel history, testing for both antibody and antiGEN
> (usually urine, but blood also depending on lab availability) for
> Blastomyces, Histoplasma, and/or Coccidioides.  If he has a substantial
> animal/farm exposure history, then this will be a bigger headache.
>
> That said, I am surprised ID did not even suggest OI prophylaxis with such
> a low CD4 count and absent mitogens.
>
> With the smoking history and the lymphopenia, lung cancer will go up the
> D/Dx ... I'll defer to the internists out there to comment more.
>
> Good luck.
>
>       - Karl
>
>
> Karl O. A. Yu, M.D., Ph.D., F.A.A.P.
> Scientist and Assistant Director, Center for Infectious Diseases and
> Immunology
> RGH Research Institute | Rochester General Hospital | Rochester Regional
> Health
> 1425 Portland Ave., Room R-403, Rochester, NY   14621
> Tel  585-922-3709  |  Fax  585-922-2415
>
>
>
>
>
>
> -----Original Message-----
> From: cis-pidd at lyris.dundee.net [mailto:cis-pidd at lyris.dundee.net] On
> Behalf Of CIS-PIDD
> Sent: Wednesday, November 22, 2017 10:52 AM
> To: CIS-PIDD
> Cc: Quinn, James M CIV USARMY MEDCOM BAMC (US)
> Subject: [cis-pidd] Asymptomatic patient with low ALC, hypogamm, CD4 count
> 17, and absent mitogen stim - management recommendations?
>
> Greetings,
>
> I have an interesting case that I would like to present to the group.  Any
> feedback is welcome regarding management recommendations.
>
> 61 yo Caucasian male with PMHx significant for allergic rhinitis, chronic
> lymphopenia and hypogammaglobulinemia without recurrent infections presents
> for follow-up. He was referred to our clinic for allergic rhinitis, at
> which
> time chart review demonstrated chronic lymphopenia. He reported a family
> history of "abnormal immune systems and rare cancers", but is unable to
> elaborate and is estranged from his family. His children are all adopted.
>
> PMHx: hypertension, basal and squamous cell carcinoma.
> Surgical history: denies
> Social history: ex-smoker (24 pack year history), monogamous with wife, no
> STDs, denies EtOH/illicits, retired but is a prior welder
> Medications: cetirizine, fluticasone proprionate nasal, montelukast,
> lisinopril, finasteride, and alfuzosin. At his visit he was feeling well
> and
> in his usual state of health.
>
> Lab summary pertaining to lymphopenia:
> ALC 400-700 since 2007
> Depressed IgG (400-500 mg/dL) and IgM (30 mg/mL) noted 2011, 2012, and 2017
> CDC with low CD3+, CD3+/CD4+, CD3+/CD8+ and percentage compensation of
> CD3-CD19+ and CD3-/CD56+
> Mitogen stim test without response mitogens or antigens (Con A, pokeweed,
> tetanus, candida, and phytohemagglutinin)
> Diptheria and tetanus titers protective
> Pneumococcal 23/23 (100%) response to vaccine
> Normal H/H and plts
> TB (t-spot) test negative
> HIV-1 viral load ultrasensitive 0 copies
> Smear without pathology noted
> CH50 elevated
> Complete metabolic panel normal
> CXR 2/2017 normal
> HRCT chest 4/2017 nonspecific air trapping; groundglass opacities with
> mosaic attenuation pattern; no bronchiectasis; multiple intrafissural LN
> measuring up to 1.2cm; RLL 7 mm nodule
> CT chest 10/2017 was without bronchiectasis but showed pulmonary nodules.
> Spirometry normal
>
> Referred to infection diseases who felt no underlying infection was causing
> his lymphopenia; they did not recommend OI prophylaxis. Referred to
> Hem/Onc,
> who did not recommend a bone marrow biopsy and did not offer follow-up. He
> denies any infection since his last visit in our clinic.
>
>
> Recommendations we made:
> 1- no live vaccines
> 2- If requires blood products recommend irradiated, CMV negative
> 3- follow-up of pulmonary nodules in 3 months with CT chest and pulmonary
> referral was placed
> 4- monitor clinically for further evaluation (given labs are already
> abnormal, low utility in "screening routinely")
>
> Are we missing something?  Would you consider anything else in your
> evaluation/management/counseling plan?  Appreciate your time and thoughts.
>
> Happy Thanksgiving,
>
> REBECCA SCHAPIRA, DO
> Capt, USAF, MC
> Fellow, Allergy/Immunology, SAUSHEC
> Assistant Professor of Medicine, USUHS
>
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