Successful treatment of severe Shulman's syndrome by allogeneic bone marrow transplantation

Bone Marrow Transplantation
P CetkovskýM Svojgrová

Abstract

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.

Citations

Jul 11, 2009·Journal of Clinical Rheumatology : Practical Reports on Rheumatic & Musculoskeletal Diseases·Rodney S DanielMarcy B Bolster
Feb 14, 2009·The American Journal of the Medical Sciences·Rodney S Daniel, Alan N Brown
Oct 9, 2012·Best Practice & Research. Clinical Rheumatology·David Lebeaux, Damien Sène
Sep 20, 2015·La Revue de médecine interne·D Sène
Feb 3, 2005·British Journal of Haematology·R E HoughN M Wulffraat
Apr 14, 2006·Mayo Clinic Proceedings·Ayalew TefferiAnimesh Pardanani
Mar 18, 2017·American Journal of Clinical Dermatology·Jorre S MertensElke M G J de Jong
Dec 14, 2017·The Journal of Dermatology·Masatoshi JinninHironobu Ihn
Feb 16, 2018·International Journal of Dermatology·Amanda F NahhasHenry W Lim

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