Successful treatment of severe Shulman's syndrome by allogeneic bone marrow transplantation
Language English Country Great Britain, England Media print
Document type Case Reports, Journal Article, Research Support, Non-U.S. Gov't
PubMed
9543071
DOI
10.1038/sj.bmt.1701137
Knihovny.cz E-resources
- MeSH
- Adult MeSH
- Eosinophilia therapy MeSH
- Fasciitis therapy MeSH
- Adrenal Cortex Hormones therapeutic use MeSH
- Humans MeSH
- Graft vs Host Disease drug therapy MeSH
- Bone Marrow Transplantation * MeSH
- Check Tag
- Adult MeSH
- Humans MeSH
- Male MeSH
- Publication type
- Journal Article MeSH
- Case Reports MeSH
- Research Support, Non-U.S. Gov't MeSH
- Names of Substances
- Adrenal Cortex Hormones MeSH
We describe a patient with severe Shulman's syndrome (ShS) (eosinophilic fasciitis). This auto-immune disease involved not only the skin and muscles, but the bone marrow as well - thereby fulfilling the criteria of severe aplastic anemia. As the disease was steroid-resistant, the patient underwent allogeneic bone marrow transplantation (BMT). Remission of ShS was achieved. Eight months later chronic GVHD developed and relapse of ShS (probably induced by GVHD) occurred. He was successfully treated with corticosteroids and the disappearance of GVHD was followed by cessation of the symptoms of ShS. At present (34 months following BMT) he is doing well and displays no signs of ShS or GVHD. This case suggests that an aggressive immunoablative preparative regimen with subsequent allogeneic BMT can result in long-lasting clinical remission of a severe auto-immune disease.
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