[PAGID] Vitiligo postBMT and right ptosis

[Berger, Melvin]

Interesting to have two cases of MG in what must be a very small denominator. What about autoimmune hemolytic anemia, which seems to be the most common autoimmune diathesis in other conditiobs such as ALPS ?    Given the association of MG with thymomas in adults, is this telling us something about the thymus in transplant recipients ?
 
Melvin Berger, M.D., Ph.D.
Adjunct 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

________________________________

From: pagid-bounces at list.clinimmsoc.org on behalf of Cowan, Mort
Sent: Thu 8/20/2009 5:50 PM
To: pagid at list.clinimmsoc.org
Subject: Re: [PAGID] Vitiligo postBMT and right ptosis



We've seen a couple of Navajo SCID (Artemis deficient) who developed vitiligo years post transplant, one was a haplo and the other a very closely matched (9/10) sibling. We've also had a child with Hurler's who developed MG after unrelated donor transplant. Both manifestations certainly represent immune dysregulation and fit in the category of graft vs host albeit rare and unusual manifestations.

 

We've tried topical therapy for the vitiligo which has not been successful. For the child with MG we used pyridostigmine bromide and immunosuppression with steroids and CSA. Eventually, the MG resolved with a slow taper off steroids but it took a long time (many months) for this to occur.

 

Morton J. Cowan, M.D.

Professor of Pediatrics

Chief, Blood and Marrow Transplant Division

UCSF Children's Hospital, Room M659

505 Parnassus Ave

San Francisco, CA 94143-1278

 

Phone: 415-476-2188

FAX: 415-502-4867

 

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________________________________

From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Nacho Gonzalez
Sent: Thursday, August 20, 2009 1:11 AM
To: pagid at list.clinimmsoc.org
Subject: [PAGID] Vitiligo postBMT and right ptosis

 

Somebody can help us with this clinical case:?

 

13 y.o. male. RAG deficiency + allogenic bone marrow transplantation (haploidentical sibbling. HLA B7, A26, A28, Cw*00, B14, B35, DR7, DR14) without complications except for extensive vitiligo, starting 8 years after transplantation. Since the last year asthenia, and since the last month right ptosis. Neurologist clinically confirms myasthenia gravis (Pending Antibodies against ACh-Receptor and Tensilon´s Test (Edrophonium)). No other signs of cGVHD. 

Questions:

These are enough criteria to consider it cGVHD (extensive skin + neuromuscular junction)?

Do you have experience with the management of myasthenia gravis postBMT?

 

Thanks.

Luis Ignacio Gonzalez Granado. Immunodeficiencies Division. Hospital 12 octubre. Madrid. Spain.



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