[CIS PIDD] [cis-pidd] CVID, hyper-eosinophilia, lymphadenopathy

[Zachary D. Jacobs, MD]

Hello all,



I am hoping for some help in a case.  He is a man with CVID who I have been
following for the last couple of years.  Here is the rundown:



·         Currently he is a 39 year-old man with CVID, diagnosed at the age
of 15.  He was  having recurrent sinopulmonary infections at the time and
responded well to IVIG when started.  Currently receives he 55 grams IV
every four weeks with troughs in the 800 – 900 mg/dl range.  He has
undetectable IgA with normal IgM in the 100-150 mg/dl range.

·         Age 26, developed AIHA.  Treated with splenectomy and
corticosteroids.  Was also noted to have persistent lymphadenopathy at that
time.  Biopsy of LN showed polymorphous lymphoid aggregrates as well as
atypical follicular hyperplasia.  There was no concern for a malignant
process based upon flow cytometry and immunohistochemistry

·         Age 32, developed ITP which was treated successfully with
corticosteroids.

·         Age 34, developed progressive dyspnea and respiratory complaints
with pulmonary lesions.  He underwent a lung biopsy via VATS at that time
and a biopsy of a nodule showed bronchiolitis with organizing
pneumonia. Biopsy of a pulmonary lymph node showed a polymorphous
population of lymphocytes, mostly T-cells mature T-cells with a normal
ratio.  The histological architecture was normal and the final diagnosis
was a reactive lymphoid hyperplasia without features of a
lymphoproliferative disorder.

·         His PFTs have remained within normal limits and until recently q
6 months CT scans were stable.

·         Nearly concurrent with his diagnosis of follicular bronchiolitis
he developed hypereosinophilia.   His absolute eosinophil count since then
has waxed waned since then but it is never less than a 1,000 cells per
microliter and upon review of his record since then it has gone as high as
4000 cells per microliter on one occasion.  His average AEC is about 2500.

o   Hypereosinophilia is moderately steroid sensitive when they are used
short term (takes AEC down to about 800).  Hydroxycholoroquine has no
effect and he has been resistant to undergo treatment with other agents
such as azathioprine.

o   Echo obtained ~ 6 months ago was normal.

o   Bone marrow biopsy obtained about six months ago showed no molecular
defects associated with hyper-eosinophilia syndrome.

·         Limited CT scan of sinuses show near complete opacification of
sinuses with nasal polyposis.

·         B-cell subset analysis shows low switched memory-B cell counts
and low CD21 expression.  CD4, CD8 and NK cell numbers were normal with
normal relative frequencies.

·         Bronchoscopy with BAL in August 2012 as well as last month showed
negative cytology, no eos, and negative cultures.

·         About six weeks ago he had increased shortness of air and
fatigue.  He went to the ER where an echo showed hyperdynamic left
ventricle function and right ventricular dilatation with moderately reduced
systolic function.  He was found to have severe pulmonary hypertension with
PA pressure 90/54.  Remodulin was started.

·         CT scans showed interval development of multiple discreet large
pulmonary nodules bilaterally predominately in the lower lobes.  He also
had interval development of mediastinal hilar lymphadenopathy.  Abdominal
scan showed stable retroperitoneal and peritoneal adenopathy.

·         PET scan showed extensive mediastinal axillary, peritoneal and
inguinal hypermetablic lymphadenopathy.  He had a dominant right lower lob
infiltrate with increased uptake.  Multifocal pulmonary nodules also had
increased FDG intake.  Nuclear med bone marrow scan showed no evidence of
extramedullary hematopoiesis.  SPECT imaging showed no evidence of abnormal
accumulation in any of the pulmonary modules.

·         Flow cytometry of peripheral blood and axillary lymph nodes
showed no evidence of monoclonality or increased blasts.

·         The biggest problem right now is an open lung biopsy cannot be
performed because of his pulmonary hypertension.  He is scheduled for
another bronchoscopy next week, at which point a transbronchial biopsy can
be obtained.



Any thoughts on these findings, especially in the context of his
longstanding hyper-eosinophilia, would be much appreciated.  Further
approaches to his diagnosis and treatment would also be
great.  Sarcoid-like disease associated with CVID would otherwise be high
on my differential because it can theoretically be associated with
eosinophilia, but prior biopsies have failed to show any granulomatous
inflammation.  ACE level, for what it’s worth, was very mildly elevated at
69 (reference range 12 – 68 U/L).



Thanks, as always, for the help.



Zach

-- 
Zachary D. Jacobs, M.D.

The Center for Allergy & Immunology

Saint Luke’s Physician Partners
Medical Plaza II
4330 Wornall, Suite 40
Kansas City, MO 64111

Ph: 816.531.0930
Fax: 816.753.2671

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