[PAGID] Patient question

[HOWARD M LEDERMAN]

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

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>  

>  -----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

>  > 

>  > 

>  > 

>  > 

>  

>