[CIS PIDD] [cis-pidd] Secondary Hypogamm to CMV with reactive T-cell monoclonality?

[Zachary D. Jacobs, MD]

Dear all,



I saw a 31 year-old woman at the consultative request of oncology for
immune evaluation.  She had been very fatigued, to the point of not being
able to get out of bed for several weeks.  This prompted the initial
oncology visit.  She had some mild pancytopenia and mildly elevated liver
transaminases, both of which have resolved.  For the past two or three
years, she also has had symptoms consistent with clinical sinusitis and
good response to antibiotics. She doesn’t have a history of invasive,
aggressive, or atypical infections.



A bone marrow biopsy was performed which was overall normal.. Cellularity
was 70%.  Flow cytometry of the bone marrow was abnormal for an inverse
ratio of CD4 to CD8 T cells.  There were also scarce to absent plasma cells
. She also had a monoclonal pattern identified on her T-cell gamma receptor
rearrangement studies.  The T-cell receptor gamma clonality could be
consistent with a T large granular lymphocytic leukemia. She also had some
atypical lymphocytes noted on the peripheral smear.



Oncology found her to be pan hypogammaglobulinemia with total IgG 316
mg/dl, IgA of 10 mg/dl and IgM of 24 mg/dl.  Serum free light chains and
SPEP immunofixation were normal. Hepatitis serology was negative. CMV PCR
was positive at 945 IU/ML. CMV log 10 was 2.975 log 10 IU/ML. EBV and
parvovirus PCR was negative. Screening ANA was negative.  HIV testing was
negative.



In my clinic, these are her labs:


   - IgG: 318 mg/dl
   - IgA: undetectable
   - IgM: 26 mg/dl
   - Tetanus antibody: 0.26 IU/mL
   - S pneumo specific antibody panel: two of the 23 tested serotypes were
   above 1.3 mcg/mL (1.4 and 1.8 mcg/mL).  Pneumovax given.
   - CBC: Mild normocytic anemia at (11), normal WBCs and Plts
   - WBC diff: Normal
   - CMP: Normal
   - Flow cytometry of peripheral blood: absolute count of CD8 cells
   increased at 1600 cells/mcl.  Inverse CD4:CD8 ratio at 0.5.  Absolute count
   of CD19 cells mildly decreased at 32 cells/mcl with relative frequency of
   B-cells at 1% total ALC.  Normal count and relative frequency of NK cells.
   Normal counts and relative frequency of switched/unswitched memory B-cells.

The oncologist is wondering if the potential monoclonal pattern seen on her
marrow T-cell receptor gamma gene rearrangement could be a reactive
non-neoplastic lymphoid proliferation secondary to a primary
hypogammaglobulinemia.  I am also wondering if her
pan-hypogammaglobulinemia could be transient secondary to CMV?  I found a
couple case reports but it seems like the CMV infections in these cases
were much more clinically severe.  Or of course is this “just” CVID
(pending confirmatory poor antibody response to PPSV23)?

Any thoughts would be much appreciated.


Thanks as always!

Zach
--
Zachary D. Jacobs, M.D.
Allergy, Asthma & Clinical Immunology

The Center for Allergy & Immunology

Kansas City Physician Partners
4330 Wornall, Suite 40
Kansas City, MO 64111

www.kcallergycenter.com

Ph: 816.531.0930
Fax: 816.753.2671
Personal e-fax: 888.283.2583

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