[CIS-PAGID] Peripheral neuropathy in CVID

[Jason Raasch, MD]

The dichotomy of our reference to immunoglobulin therapy is interesting:  to third-party payors, pharmacies, etc. we preach the importance of having a choice for our patients as there are clear differences among products.  Yet in our academic discussions and communications we simply refer to it as "IVIG" or "SQ" (as if mentioning a brand is irrelevant or a delicate subject).

In patients who have apparent reactions to immunoglobulin therapy I think we must discuss the specific preparations, not just the route.  

Howard, it would be of interest to know how long the first patient you mentioned was on Hizentra before the onset of neuropathy symptoms and if this patient had been transitioned to it from IVIG (and which one!).  Same thing for the out-of-town patient referred to.

Agree with the question Richard poses with regard to Privigen, as a common denominator between it and Hizentra is proline.  Sweden and UK data may suggest SQ vs IV (ROUTE!) is not the issue, but what about specific product?  I admit I cannot quote specifics of these studies - I'll get back to you on that after I review.

Perhaps related to this is Joe's patient with presumed mitochondrial disorder.  The key enzymes required for proline metabolism are proline dehydrogenase and P5C, both located in the mitochondria.  Could the neuropathy be related to proline toxicity (from Hizentra) secondary to the mitochondrial disorder (dysregulated proline metabolism)?  Yes, this is a stretch but I again argue that these are the types of questions we need to ask.

Agree with Marc  that in that these patients  with apparent difficulty tolerating Hizentra, it IS important to try to switch to the other SQ products.  I have two patients who tolerated Vivaglobin well.  Flu-like symptoms with hizentra (that admittedly I did not believe for a while).  Tried SQ Gamunex with similar symptoms.  Transitioned to SQ Gammagard and both have tolerated as well as Vivaglobin.  Switched BACK to Hizentra (these were very willing patients) and flu-like symptoms appeared again.

Kate's concern is valid and a call to discuss and study not just SQ vs IVIG (ROUTE!) but also Gammagard vs Privigen vs Gammaplex vs [all others].  Yes, this would be difficult - but what hasn't been in clinical immunology?



-Jason  


Jason Raasch, MD

Midwest Immunology Clinic
15700 37th Ave N
Ste 110
Plymouth, MN 55446

Phone: (763) 577-0008
FAX:     (763) 577-0192



On Apr 18, 2012, at 14:31 PM, Howard Lederman wrote:


> I have an adult CVID patient who has developed an idiopathic peripheral neuropathy while on Hizentra.  My first reaction was to think that this was merely a coincidence and not cause and effect.  However, yesterday I received a call about an out-of-town patient who had CVID, had recently been switched from IVIG to Hizentra and then  developed parasthesias.

>  

> Do any of you have patients with similar histories?  

>  

> Howard

>  

>  

> Howard M. Lederman, M.D., Ph.D.

> Professor of Pediatrics, Medicine and Pathology Division of Pediatric Allergy and Immunology Johns Hopkins Hospital - CMSC 1102 600 N. Wolfe Street Baltimore, MD 21287-3923

> Phone: 410-955-5883

> Fax: 410-955-0229

> Email: Hlederm1 at jhmi.edu

>  

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