Biomaterial Database

Successful treatment of severe Shulman's syndrome by allogeneic bone marrow transplantation. 1998

P Cetkovský, and V Koza, and P Cetkovská, and M Svojgrová

Department of Haematology and Oncology, Charles University Hospital, Pilsen (Plzen), Czech Republic.

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.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D004802	Eosinophilia	Abnormal increase of EOSINOPHILS in the blood, tissues or organs.	Eosinophilia, Tropical,Hypereosinophilia,Tropical Eosinophilia,Hypereosinophilias,Tropical Eosinophilias
D005208	Fasciitis	Inflammation of the fascia. There are three major types: 1, Eosinophilic fasciitis, an inflammatory reaction with eosinophilia, producing hard thickened skin with an orange-peel configuration suggestive of scleroderma and considered by some a variant of scleroderma; 2, Necrotizing fasciitis (FASCIITIS, NECROTIZING), a serious fulminating infection (usually by a beta hemolytic streptococcus) causing extensive necrosis of superficial fascia; 3, Nodular/Pseudosarcomatous /Proliferative fasciitis, characterized by a rapid growth of fibroblasts with mononuclear inflammatory cells and proliferating capillaries in soft tissue, often the forearm; it is not malignant but is sometimes mistaken for fibrosarcoma.	Fascitis,Fasciitides,Fascitides
D006086	Graft vs Host Disease	The clinical entity characterized by anorexia, diarrhea, loss of hair, leukopenia, thrombocytopenia, growth retardation, and eventual death brought about by the GRAFT VS HOST REACTION.	Graft-Versus-Host Disease,Homologous Wasting Disease,Runt Disease,Graft-vs-Host Disease,Disease, Graft-Versus-Host,Disease, Graft-vs-Host,Disease, Homologous Wasting,Disease, Runt,Diseases, Graft-Versus-Host,Diseases, Graft-vs-Host,Graft Versus Host Disease,Graft-Versus-Host Diseases,Graft-vs-Host Diseases
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000305	Adrenal Cortex Hormones	HORMONES produced by the ADRENAL CORTEX, including both steroid and peptide hormones. The major hormones produced are HYDROCORTISONE and ALDOSTERONE.	Adrenal Cortex Hormone,Corticoid,Corticoids,Corticosteroid,Corticosteroids,Cortex Hormone, Adrenal,Hormone, Adrenal Cortex,Hormones, Adrenal Cortex
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults
D016026	Bone Marrow Transplantation	The transference of BONE MARROW from one human or animal to another for a variety of purposes including HEMATOPOIETIC STEM CELL TRANSPLANTATION or MESENCHYMAL STEM CELL TRANSPLANTATION.	Bone Marrow Cell Transplantation,Grafting, Bone Marrow,Transplantation, Bone Marrow,Transplantation, Bone Marrow Cell,Bone Marrow Grafting