[PAGID] Vitiligo postBMT and right ptosis

[Cowan, Mort]

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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