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

Wed Nov 22 13:12:11 EST 2017

I had a similar patient who we thought had recalcitrant histoplasmosis of the lung and idiopathic cd4 lymphopenia at age 31. Turned out he had abnormal lymphatics around his lungs (kidneys too but that had minimal consequences). He had a 2 decade history of productive cough that he ignored. He had an abnormal CT chest (incidentally noted when he had an abdominal CT for another reason). After  18 months of treatment for presumed pulmonary histoplasmosis, Lung biopsy showed desquamative interstitial pneumonitis. Subsequently, lymphoscintigraphy showed his lymph poured into a left sided lymphatic malformation in his chest with a fistula to his left lower lobe of his lungs. He eventually went for Thoracic duct  embolization which was successful.
Despite CD4 counts of 12-60 (with 4% naive only!) and low mitogens when we assessed prior to embolization, he was perfectly healthy otherwise.
Kimberly Risma MD PhD
Associate Professor Pediatrics
Cincinnati Children’s Hospital Medical Center

Sent from my iPhone

On Nov 22, 2017, at 11:58 AM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> wrote:

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<mailto: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<mailto: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

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From: cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net> [mailto: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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