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

[Zachary D. Jacobs, MD]

Thanks to everyone so far for the very helpful information.  The
hyper-eosinophilia along with a relatively stable follicular bronchiolitis
has been present for the past seven years or so.  I have only been
following him for the past two years, and my work-up has been hampered by
the fact that getting him to agree to any study is like pulling teeth.  He
felt well so didn't want to do anything about it. It has only been the past
several months where I have been able to start to piecemeal together on him
a more complete work-up and everything has accelerated in the past weeks
with the development of the pulmonary hypertension.  I have not tested for
sIL2R.  Where would I go for sequencing of PIK3CD?

Thanks,

Zach


On Thu, Dec 12, 2013 at 12:50 AM, Klaus Warnatz <
klaus.warnatz at uniklinik-freiburg.de> wrote:


> HI,

>

> I agree with what has been stated before. granulomatous disease may not be

> evident on biopsy and possibly also not present in ILD in your patient.

> Do you have a follow up of sIL2R ? did I understand correctly that the

> eosinophilia developed two years ago? If you are replanning bronchoscopy

> anyway I would check again for pathogens and for eosinophils in the BAL.

> Given the increased CD21low cells I would consider RTX because we find

> these cells often in the BAL of these patients (obviously not knowing

> whether these cells are part of the pathophysiology). Steroids I would also

> use.

> Have you considered PI3K def in the patient?

> I also think that this patient runs a higher risk for lymphoma.

>

> best regards (could you give us an update on the further course, please?)

>

> klaus

> Prof. Dr. med. Klaus Warnatz

>

> UNIVERSITÄTSKLINIKUM FREIBURG

> University Medical Center Freiburg

> Center for Chronic Immunodeficiency

> Division of Rheumatology and Clinical Immunology

>

> Tel: +49-761-270-77640 / FAX -71000 / Pager: 12-7100

>

> Breisacher Str. 117, 79106 Freiburg, Germany

> klaus.warnatz at uniklinik-freiburg.de

> http://www.uniklinik-freiburg.de/cci

>

> Am 11.12.2013 um 21:55 schrieb Verbsky, James:

>

> Zach

>

> I think you are dealing with GLILD (sarcoid like lung disease in CVID).

> Follicular bronchiolitis, lymphocytic interstitial pneumonia, and

> granulomatous disease can all be seen in this.  Actually Charlotte

> Cunningham-Rundles last series looking at lung pathology in CVID did not

> show granulomas.  My guess is there is some sampling error or clinical

> variability, but I would not let the lack of granuloma skew away from this

> diagnosis.  Treatment is controversial.  Jack Routes has a small series on

> Rituxan/Imuran.  Charlottes paper had a case of rituxan.  Others have used

> cellcept.  I would argue this person needs more than steroids.

> Best

>

> James

>

>

> James W. Verbsky M.D./Ph.D.

> Associate Professor of Pediatrics and Microbiology

> Medical College of Wisconsin

> Milwaukee, WI

> 414-266-6701

>

>

>

> *From:* Zachary D. Jacobs, MD [mailto:zjacobs.md at gmail.com]

> *Sent:* Wednesday, December 11, 2013 10:29 AM

> *To:* CIS-PIDD

> *Subject:* [cis-pidd] CVID, hyper-eosinophilia, lymphadenopathy

>

> 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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-- 
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

 CONFIDENTIALITY NOTICE. This e-mail and attachments (if any) may contain
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