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

[CIS-PIDD]

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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