[PAGID] Patient question

[Cowan, Mort]

I agree. We rule out hepatitis B in our patients first.

Morton J. Cowan, M.D.
Professor of Pediatrics
Chief, BMT Division
UCSF Children's Hospital, Room M659
505 Parnassus Ave
San Francisco, CA 94143-1278
 
Phone: 415-476-2188
FAX: 415-502-4867
 
**Confidentiality Notice** This email communication and any attachments
may contain confidential and privileged information for the use of the
designated recipients named above. Distribution, reproduction or any
other use of this transmission by any party other than the intended
recipient is prohibited.

-----Original Message-----
From: pagid-bounces at list.clinimmsoc.org
[mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of HOWARD M
LEDERMAN
Sent: Tuesday, June 26, 2007 1:14 PM
To: pagid at list.clinimmsoc.org
Subject: Re: [PAGID] Patient question

We have also had good experience with Rituxan in our CVID pts who have
immune cytopenias.  There's probably little concern about B cell
suppression in a pt getting IVIG.  However, I think that rituxan has
been associated with recurrence of hepatitis B, so I would be wary about
using it in someone who has a past hx for that.

Howard
Howard M. Lederman, M.D., Ph.D.
Professor of Pediatrics and Medicine
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
e-mail: Hlederm1 at jhem.jhmi.edu


----- Original Message -----
From: "Cowan, Mort" <mcowan at peds.ucsf.edu>
Date: Tuesday, June 26, 2007 3:27 pm
Subject: Re: [PAGID] Patient question
To: pagid at list.clinimmsoc.org



> We start steroids on our post BMT AIHA patients but add rituxamab

fairly

>  soon as it appears to be steroid sparing. We've also found that it

takes

>  a while to kick in so it doesn't replace steroids, at least in our

>  hands.

>  

>  Mort

>  

>  Morton J. Cowan, M.D.

>  Professor of Pediatrics

>  Chief, BMT Division

>  UCSF Children's Hospital, Room M659

>  505 Parnassus Ave

>  San Francisco, CA 94143-1278

>   

>  Phone: 415-476-2188

>  FAX: 415-502-4867

>   

>  **Confidentiality Notice** This email communication and any

attachments

>  may contain confidential and privileged information for the use of

the

>  designated recipients named above. Distribution, reproduction or any

>  other use of this transmission by any party other than the intended

>  recipient is prohibited.

>  

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

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

>  [ On Behalf Of Notarangelo,

>  Luigi

>  Sent: Tuesday, June 26, 2007 7:40 AM

>  To: pagid at list.clinimmsoc.org

>  Subject: R: [PAGID] Patient question

>  

>  I can only comment re: strategy to treat AIHA. While we continue to

use

>  steroids as firts choice, we would then definitely follow with

rituximab

>  if needed, and avoid splenectomy.

>  Of several severe cases of AIHA that I have seen during the last few

>  years (mostly after HSCT), none has required splenectomy.

>  

>  Luigi Notarangelo

>  Professor of Pediatrics, Harvard Medical School

>  Division of Immunology, Children's Hospital

>  Karp Research Building, 9th floor, Rm 09210

>  1 Blackfan Circle

>  Boston, MA 02115

>  USA

>  tel: 617-9192277

>  

>  

>  

>  -----Messaggio originale-----

>  Da: pagid-bounces at list.clinimmsoc.org per conto di Berger, Melvin

>  Inviato: ven 22/06/2007 18.02

>  A: pagid at list.clinimmsoc.org

>  Oggetto: RE: [PAGID] Patient question

>   

>  I have starting hearing anecdotes about Rituxin in RA. I have not

>  actually read these papers but am attaching a couple of abstracts on 

> its

>  use in a situation that sounds somewhat like your pt. We have been

>  extremely impressed with its efficacy and lack of adverse effects in

>  CVID patients with antibody-mediated cytopenias, etc. 

>  

>  As a separate question actually, I am wondering whether we should

still

>  go through the process of   inducing remission (for example, for

severe

>  Autoimmune Hemolytic Anemia in CVID)with steroids, then doing a

>  splenectomy, vs just giving rituxin right off and leaving the spleen 

> in.

>  

>  

>  Does anyone have any idea what might be the relative effects on

future

>  incidence of lymphoma ? 

>  

>  Melvin Berger, M.D., Ph.D.

>  Professor of Pediatrics and Pathology

>  Case Western Reserve University

>  phone 216 844 3237

>  

>  Director, Jeffrey Modell Center for Primary Immune Deficiencies

>  Division of Allergy-Immunology

>  Rainbow, Babies and Children's Hospital

>  University Hospitals of Cleveland

>  RB&C Rm 504, MS 6008B

>  11100 Euclid Ave.

>  Cleveland, OH 44106

>  

>  

>  

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

>  From: pagid-bounces at list.clinimmsoc.org on behalf of Chris Seroogy

>  Sent: Fri 6/22/2007 10:42 AM

>  To: pagid at list.clinimmsoc.org

>  Subject: Re: [PAGID] Patient question

>   

>  Thank you, Mary Ellen.  Here is some more information:

>  

>  1. FISH was done for 22q11 deletion and was negative by report (I

will

>  reconfirm that.)

>  2. CVID diagnosis based on absent IgA and poor polysaccharide

response

>  (pneumovax, although isohemagglutins fair at 1:8), normal T cell

numbers

>  and

>  function.  IgM low at 32 and patient has been on IVIG for 3 years for

>  the

>  laboratory values and recurrent sinusitis.  So perhaps this diagnosis



> is

>  "soft."

>  3. Allergy to insulin and b-lactams (desensitized to both and she has

>  dexamethasone in her insulin pump.)

>  4. Several autoimmune problems:  type I DM, hypothyroidism

>  5. Chronic abdominal pain with ongoing GI evaluation.

>  6. Eczema

>  7. Factor V Leiden deficiency

>  

>  She is (was) a very active girl and highly intelligent with many

>  talents.

>  Her growth has been okay.

>  

>  Regarding infectious work-up, she had EBV,CMV, HIV, toxo ruled out by

>  PCR or

>  antibody testing.  Chest, sinus and abdominal CT with contrast

>  unrevealing ,

>  bone scan negative.  The possibility of an infection was raised by

>  another

>  one of our colleagues and I am looking into having one of the joints

>  scoped

>  for fluid and synovial tissue if possible for a more definitive

>  diagnosis.

>  Is enterovirus a possibility and would PCR on the joint fluid provide

>  the

>  highest yield?

>  

>  I appreciate any insights/comments.  Regards, Chris

>  

>  

>  

>  

>  

>  On 6/22/07 9:02 AM, "Conley, Mary-Ellen"

<maryellen.conley at STJUDE.ORG>

>  wrote:

>  

>  > Hi Chris,

>  > I think it might help to have a little more information about the

>  patient.  A

>  > significant proportion of the patients with Pierre-Robin sequence

have

>  > abnormalities of chromosome 22q11.  Do you know if this has been

>  evaluated?

>  > How is her growth and development?  Is she active?  What are the

>  immunologic

>  > findings that support the diagnosis of CVID (serum immunoglobulins;

>  lymphocyte

>  > cell surface markers; signs of autoimmune disease etc)?

>  > 

>  > I am not sure that I would discard infectious etiologies just yet.

>  > 

>  > Mary Ellen

>  > 

>  > 

>  > 

>  > 

>  > Mary Ellen Conley, MD

>  > Department of Immunology

>  > St. Jude Children's Research Hospital

>  > 332 N. Lauderdale

>  > Memphis, TN 38105-2794

>  > FAX  901-495-3977

>  > TEL  901-495-2576

>  > 

>  > 

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

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

>  > [ On Behalf Of Chris Seroogy

>  > Sent: Thursday, June 21, 2007 6:58 AM

>  > To: pagid at list.clinimmsoc.org

>  > Subject: [PAGID] Patient question

>  > 

>  > Dear Colleagues,

>  > 

>  > I have a rheumatologic question on a very complicated CVID patient 

> and

>  am

>  > hoping some of you have faced this management challenge before.

This

>  is a

>  > 13y/o female with long-standing brittle type I DM, CVID (mostly

>  functional

>  > antibody production problems and IgA deficient), Pierre-Robin

>  sequence, and

>  > multiple drug allergies.  She presented to my clinic for second

>  opinion re:

>  > CVID management last Fall and at that time I noted significant

>  arthritis is

>  > several large joints and synovitis was confirmed by MRI and several

>  bony

>  > erosions were seen on plain xray.  Since this time, I have been

>  treating her

>  > arthritis (seronegative) aggressively with poor response.  She is

on

>  MTX and

>  > humira, low dose daily prednisone.  She did not respond to Enbrel

for

>  12

>  > weeks, hence was switched to humira.  She receives IVIG every 3

weeks

>  (she has

>  > problems with recurrent sinusitis.) Despite this management, she

has

>  had

>  > significant progression of her arthritis now involving most joints 

> and

>  daily

>  > pain.  I am not sure where to go next (thoughts are anakinra, bolus

>  > steroids--although challenging with her DM.)  She was seen by our

ID

>  group and

>  > her clinical picture/findings were not felt to be secondary to an

>  infection.

>  > She also had a negative bone scan.  I welcome any thoughts.

>  > Regards, Chris

>  > 

>  > 

>  > Chris Seroogy, M.D.

>  > 

>  > Assistant Professor

>  > 

>  > Dept. of Pediatrics

>  > 

>  > Mail:  H4/474 CSC, Mailstop 4108

>  > 

>  > Shipping:  H4/431 CSC, Mailstop 4108

>  > 

>  > 600 Highland Ave.

>  > 

>  > Madison, WI  53792

>  > 

>  > phone: 608- 263-2652

>  > 

>  > fax: 608-265-0164

>  > 

>  > 

>  > 

>  > 

>  

>