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

[Yeşim Yılmaz Demirdağ]

Hi,
have you considered empirically treating for strongloides and toxocara?
Also, splenectomized patients may develop abnormal eosinophilic response.
Yesim

Yesim Yilmaz Demirdag, MD
Assistant Professor of Pediatrics
Columbia University Medical Center
3959 Broadway 107N, New York, NY 10032
phone: (212) 305 2300
yyd2101 at columbia.edu


On Wed, Dec 11, 2013 at 11:28 AM, Zachary D. Jacobs, MD <
zjacobs.md at gmail.com> wrote:


> 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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