[CIS-PAGID] CVID + LIP

[Jack Bleesing]

Lagging behind this discussion a bit, couple of questions (mainly out
of feeling unsure about what to do, especially with young patients):
 
- what is the pathology that we are treating? The patients we have seen
typically have a mixture of T-cells, B-cells and plasma cells in these
lesions, such that I don't see the complete logic in combining imuran
with rituximab. Using a T-cell agent +  B-cell (or plasma cell) agent
seems to make more sense to me (after perhaps induction with short-term
steroids).
 
- once diffusion abnormalities have occurred, are these reversible? It
would make more sense to me to treat early - as per the "Warnatz/Ehl"
approach, when ritux or steroids/MMF may be enough to reverse the
disorder? Why not rituximab/MMF, as these patients are already on IVIG
and they may be better off being a CVID patient with no B-cells than a
CVID patient with "renegade" B-cells (keeping the steroids in reserve).
 
- Is HHV-7 and/or HHV-8 routinely included in the diagnostic workup and
if found in the lungs, does anybody treat?
 
- If "our" approach would be 2 treatment arms, rather than a
placebo-controlled approach, could we consider a cross-over design
(following evaluation of bench marks - imaging, BAL, PFTs. etc.)
 
- are kids with CVID (+ lung disease) different than the adults with
this? Feel much more alarmed about our young patients with pulmonary
complications, and have a hard time just watching them, or defining an
endpoint of therapy.
 
- would love to be part of a CVID workshop, where things are discussed
(as well as uniform diagnosis and agreement about definitions of LIP/
pulmonary complications, agreement about workup/benchmarks and hashing
out treatment options).
 
Regards,
 
JB
 
---------------------------------------------------------------------------
Jack J.H. Bleesing, M.D., Ph.D.
Associate Professor of Pediatrics
Cincinnati Children's Hospital Medical Center
Division of Bone Marrow Transplantation & Immune Deficiency
3333 Burnet Avenue, MLC 7015
Cincinnati, OH 45229
513-636-4266 (phone)
513-636-3549 (fax)
Jack.Bleesing at CCHMC.org 
http://www.cincinnatichildrens.org/immunodeficiencies/ 


>>> "Routes, John" <jroutes at mcw.edu> 7/25/2011 11:44 AM >>>

Elie
I think we need to get all interested parties together either in person
or
by conference call to discuss. As we have had pretty good experience
with
several patients using rituxin with imuran, it makes sense to have that
as
one arm--plus If the patient does not tolerate imuran, we can
substitute MMF
or another agreed upon drug-------the other arm is totally open and
personally would favor an arm without imuran----
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: 414-456-4802; 414-266-6997
Fax: 414-456-6487 (Clinical)
Fax: 414-456-6323 (Laboratory)
Email: jroutes at mcw.edu 





> From: Elie Haddad <elie.haddad at umontreal.ca>

> Reply-To: "pagid at list.clinimmsoc.org" <pagid at list.clinimmsoc.org>

> Date: Mon, 25 Jul 2011 09:56:30 -0500

> To: "pagid at list.clinimmsoc.org" <pagid at list.clinimmsoc.org>

> Subject: Re: [CIS-PAGID] CVID + LIP

> 

> Dear all,

> what do you think about comparing Rituximab to something else than

Rituximab.

> Indeed, in the proposed protocol, Rituximab is in both arms meaning

that we

> consider Rituximab as the gold standard therapy for this condition...

I am a

> pro RTX in this condition but maybe there are other options, as

outlined by

> Klaus.

> I would suggest to compare RTX to Steroids alone or also another

possibility

> would be to compare RTX to Infliximab.

> Also, if finally we stay with the idea of RTX in both arms, what

about MMF

> instead of Imuran ?

> Yours

> Elie

> 

> 

> 

> Elie Haddad, MD, PhD;

> Professor of Pediatrics, University of Montr

eal,

> Head, Pediatric Immunology and Rheumatology Division,

> CHU Sainte-Justine, 3175 Cote Sainte-Catherine

> Montreal, QC, H3T 1C5, Canada

> Ph: 1 514 345 4713

> fax: 1 514 345 4897

> e-mail: elie.haddad at umontreal.ca 

> 

> 

> 

> 

> Le 2011-07-22 à 17:38, David Rawlings a écrit :

> 

>> As Jack and I have discussed previously, our group in Seattle would

be keen

>> to participate in a trial- I suggest designing a 2 arm protocol eg

Ritux

>> with or without Aza.

>> David

>> 

>> -----Original Message-----

>> From: pagid-bounces at list.clinimmsoc.org 

>> [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Nelson,

Robert P Jr

>> Sent: Friday, July 22, 2011 11:15 AM

>> To: pagid at list.clinimmsoc.org 

>> Subject: Re: [CIS-PAGID] CVID + LIP

>> 

>> What about considering a mutlicenter clinical trial so we could

contibute

>> our patients to an organized effort?  Bob

>> ________________________________________

>> From: pagid-bounces at list.clinimmsoc.org

[pagid-bounces at list.clinimmsoc.org] 

>> on behalf of Fleisher, Thomas (NIH/CC/DLM) [E] [TFleishe at cc.nih.gov]



>> Sent: Friday, July 22, 2011 10:32 AM

>> To: Routes, John; pagid at list.clinimmsoc.org 

>> Subject: Re: [CIS-PAGID] CVID + LIP

>> 

>> Sorry for copying all on a simple question to Jack.

>> 

>> Thomas A. Fleisher, M.D.

>> Chief, Department of Laboratory Medicine

>> NIH Clinical Center

>> 301 496-5668 (T)

>> 301 402-1612 (F)

>> 

>> -----Original Message-----

>> From: Routes, John [mailto:jroutes at mcw.edu] 

>> Sent: Friday, July 22, 2011 8:32 AM

>> To: pagid at list.clinimmsoc.org 

>> Subject: Re: [CIS-PAGID] CVID + LIP

>> 

>> We have treated over ten patients with combination chemotherapy

consisting

>> of rituxin and azathioprine with outstanding results no infections

and in

>> many cased complete or near complete clearing.

>> 

>> Sent from my Verizon Wireless BlackBerry

>> 

>> ________________________________

>> From: Terri Tarrant <tarra002 at gmail.com>

>> Sender: "pagid-bounces at list.clinimmsoc.org"

>> <pagid-bounces at list.clinimmsoc.org>

>> Date: Fri, 22 Jul 2011 06:16:47 -0500

>> To: pagid at list.clinimmsoc.org<pagid at list.clinimmsoc.org>

>> ReplyTo: "pagid at list.clinimmsoc.org" <pagid at list.clinimmsoc.org>

>> Subject: Re: [CIS-PAGID] CVID + LIP

>> 

>> Yes, we have 3 cases that responded well (2 with CVID and 1

idiopathic LIP).

>> We used the Eisenberg protocol of 1 g IV day 0 and day 14 with 100

mg IV

>> solumedrol pre-med.  We did not use in combination with other drugs.

 We did

>> not see an increase in infections.  All patients needed redosing at

9-12

>> month intervals.

>> 

>> Terri Tarrant

>> 

>> On Thu, Jul 21, 2011 at 10:07 PM, Church, Joseph

>> <JChurch at chla.usc.edu<mailto:JChurch at chla.usc.edu>> wrote:

>> Colleagues:

>> 

>> A 60yo female with long term CVID has had lymphoid interstitial

pneumonitis

>> that was biopsy documented in 2005.

>> 

>> She had developed worsening of symptoms over the past 6 months.

>> 

>> She is trying to avoid increasing her prednisone, currently at

10mg/d,

>> because of coronary artery disease and hypertension; she could not

tolerate

>> CellCept.

>> 

>> Has anyone tried Rituximab in this setting?

>> 

>> Joe Church, MD

>> Children's Hospital Los Angeles

>> 

>> 

>> 

>>

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>> 

>> 

>> 

>> --

>> Terri Tarrant, MD

>> Assistant Professor of Medicine

>> Thurston Arthritis Research Center

>> Lineberger Cancer Center Member

>> CB # 7280, 3300 Manning Dr.

>> Chapel Hill, NC 27599

>> (919) 843

-4727

>> http://tarc.med.unc.edu/tarrant_welcome.php 

>> 

>> 

>> 

>> 

> 


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