[PAGID] Vitiligo postBMT and right ptosis

[Nacho Gonzalez]

Thanks for your answers,
the patient with MG and vitiligo postBMT is improving with piridostigmine
and steroids(Pred 1mg/kg/day).
The neurologist says like Melvin, what is about thymus in trasplant
recipients? *Has anybody performed thymectomy in MG postBMT?  *

Thanks again for your help

Nacho Gonzalez. Immunodeficiencies Unit. Hospital 12 de octubre. Madrid.
Spain

2009/8/21 Berger, Melvin <Melvin.Berger at uhhospitals.org>


> 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.

>

>

>

> Visit us at www.UHhospitals.org <http://www.uhhospitals.org/>.

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