Graves' orbitopathy in a patient with adrenoleukodystrophy after bone marrow transplantation.
OBJECTIVE
For many years, the treatment of X-linked childhood cerebral adrenoleukodystrophy (XALD) consisted of hydrocortisone replacement and a mixture of short chain-fatty acids, known as 'Lorenzo's oil'. Recently, bone marrow transplantation (BMT) has also been used.


METHODS
We report the case of a patient affected by XALD who developed Graves' hyperthyroidism (GH) and Graves' orbitopathy (GO) after BMT and who we could follow-up for 6.5 years afterwards.


RESULTS
A boy affected by XALD was treated at the age of 6 years, with a whole BMT from his sister. One year after BMT, the transplanted patient presented TSH at the lower normal value and 3 years later he developed thyrotoxicosis. After a further 2 years, the patient developed GO, which showed clinical evidence of reactivation 5 years after its onset as a consequence of an attempt to treat thyrotoxicosis by means of I(131) (300 MBq). Seven years after BMT, the donor showed alterations of thyroid autoimmunity and 1 year thereafter she developed GH. She never presented GO during a subsequent 5 year follow-up.


CONCLUSIONS
This case illustrates that autoimmunity originating from a pre-symptomatic donor can be transferred into the host during allogeneic stem cell transplantation. In cases where autoimmune phenomena are recognized in the donor prior to donation, alternative donors or T-cell manipulation of the graft might be considered.
Vardizer
Y
Y
Department of Ophthalmology, Orbital Center, University of Amsterdam, Room D2-436, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands.
Lupetti
A
A
Vandelanotte
S
S
Lankester
A C
AC
Wiersinga
W M
WM
Baldeschi
L
L
D000326:Adrenoleukodystrophy / Q000276:immunology* / Q000601:surgery; D016026:Bone Marrow Transplantation / Q000009:adverse effects / Q000276:immunology*; D002648:Child; D005260:Female; D006111:Graves Disease / Q000209:etiology / Q000276:immunology*; D006801:Humans; D008297:Male; D013972:Thyrotropin / Q000097:blood
D013972:Thyrotropin