[CIS PIDD] [cis-pidd] Renal transplantation in CVID

Mon Jun 8 18:09:18 EDT 2015

Thanks so much to Bodo Grimbacher, Mary Hogan, & John Routes for your very helpful replies. 

I think Jack's correct about the liver. I certainly assumed it was nodular regenerative hyperplasia based on my reading of this liver complication in CVID (Ward et al Clin & Exp Immunology: 2008, 153, 331-7, Fuss et al J. Clin Immunol 2013, 33(4) 748-58). The actual liver histology report described granulomatous inflammation with granulomas in the portal tract accompanied by an infiltrate of lymphocytes, histiocytes and eosinophils. There were necro-inflammatory foci in the lobule, also with inflammatory infiltrates. Reticulin stain showed foci of "focal nodular hyperplasia" with nodules of hyperplastic hepatocytes with liver cell plates two cells thick. Bile Sirius Red stain shows extensive pericellular fibrosis in zone 3. The inflamed portal tracts show fibrous expansion.

So this fits NRH rather than FNH? And needs more aggressive immunosuppressive therapy? 

Thanks so much again.

Stan
-------------------------------------------------------------------------------------
Stanley Ress
Emeritus Associate Professor of Medicine, UCT
Specialist physician & Clinical Immunologist
UCT Private Academic hospital,
Anzio Road, Observatory,
Cape Town, 7925
South Africa
TEL:INTERN. + 2721-4421966 or 4421816
FAX:   "    + 2721-(0)865173095
Cell: 0833115482 
email: stan.ress at uct.ac.za

-----Original Message-----
From: Routes, John [mailto:jroutes at mcw.edu] 
Sent: 08 June 2015 10:06 PM
To: CIS-PIDD
Subject: Re: [cis-pidd] Renal transplantation in CVID

Stan
Are you sure the liver pathology wasn't nodular regenerative hyperplasia-certainly has the clinical features of this? If so, then immunosuppressives would be needed as this is increasingly recognized as a potentially fatal complication of CVID Jack

John M. Routes, MD
Chief, Section of Allergy and Clinical Immunology Professor of Pediatrics, Medicine, Microbiology and Molecular Genetics Department of Pediatrics Children's Hospital of Wisconsin Medical College of Wisconsin
9000 W. Wisconsin Ave.
Milwaukee, WI  53226-4874
Phone: Office 414-266-6840
Fax: 414-955-6487 (Clinical)
Fax: 414-955-6323 (Laboratory)
Email: jroutes at mcw.edu<mailto:jroutes at mcw.edu>

From: Bodo Grimbacher <bodo.grimbacher at uniklinik-freiburg.de<mailto:bodo.grimbacher at uniklinik-freiburg.de>>
Reply-To: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Date: Monday, June 8, 2015 at 2:59 PM
To: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Subject: Re: [cis-pidd] Renal transplantation in CVID

Dear Stan,
We have many patients with CVID and autoimmune features on immunosupressive treatment.
If you take care of good trough levels and give antibiotics early and long enough, the bronchiectatic lung disease may not progress at all.
Immunosupression may actually help the granulomatous complications seen in your patient.
So it is a clear option for your patient.
We do prefer steroids and MMF over the other immunosupressants though. Best, Bodo
****************************************
Univ.-Prof. Dr. med. B. Grimbacher

Scientific-Director
CCI-Center for Chronic Immunodeficiency
UNIVERSITÄTSKLINIKUM FREIBURG
Tel.: 0761 270-77731  Fax: -77744
Engesserstraße 4, 79108 Freiburg
bodo.grimbacher at uniklinik-freiburg.de<mailto:bodo.grimbacher at uniklinik-freiburg.de>
www.uniklinik-freiburg.de/cci

Von: Stan Ress <stan.ress at uct.ac.za<mailto:stan.ress at uct.ac.za>>
Antworten an: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Datum: Monday 8 June 2015 21:42
An: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Betreff: [cis-pidd] Renal transplantation in CVID

Dear Colleagues,

I would be grateful for advice regarding further management in a complicated case of CVID.

A 35 year-old lady was diagnosed with CVID in 2008 and commenced on IVIG. She has had a complicated course. In 2010  a chest CT scan showed bilateral bronchiectasis as a result of recurrent RTI's. She then developed hepatomegaly and progressive splenomegaly. Bone marrow trephines done at another centre in 2008 & 2010 showed normal T-and B-cell gene rearrangements, but she developed severe bicytopenia due to hypersplenism, and eventually required splenectomy in April 2014 when splenic size was 24 cm, platelet count < 80, WCC  < 1.5. US and MRI of liver indicated increased porta-venous shunting of blood with portal hypertension, liver biopsy reported features compatible with focal nodular hyperplasia (FNH) with T-cell infiltration but no B-cells present. Liver enzymes then progressively increased post-splenectomy (alkaline phosphatase & gamma GT especially), this has improved on low dose prednisone. However, her renal function had also deteriorated and simultaneous renal biopsy (at the time of splenectomy)  was reported as showing interstitial nephritis.

She is managed on SC Immunoglobulin replacement, her current renal function has been stable with serum urea of 22 mmol/L  (2.8-7.2), creatinine of 371 umol/L (estimated GFR of 12 ml/min). Her nephrologist is exploring the possibility of a live related renal transplant and asks about the experience in this regard. While the QOL on haemodialysis would be less good than following a renal transplant, my concern is for the effect of immune suppression on an already immunocompromised patient who also has bronchiectasis.

We would appreciate any advice and would also be grateful to hear of any other experience with regard to transplantation in CVID patients.

Many thanks.

Stan
-------------------
Stanley Ress
Emeritus Associate Professor of Medicine, UCT Specialist physician & Clinical Immunologist UCT Private Academic hospital, Anzio Road, Observatory, Cape Town, 7925 South Africa TEL:INTERN<tel:INTERN>. + 2721-4421966 or 4421816
FAX:   "    + 2721-(0)865173095
Cell: 0833115482
email: stan.ress at uct.ac.za<mailto:stan.ress at uct.ac.za>

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