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

[Stan Ress]

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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