[PAGID] R: Patient question

[Notarangelo, Luigi]

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

> [mailto: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

> 

> 

> 

> 




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