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Autologous stem cell transplantation in a patient with severe systemic sclerosis

dc.contributor.authorVaz, C.
dc.contributor.authorAlmeida, I.
dc.contributor.authorGuedes, M.
dc.contributor.authorRosário, C.
dc.contributor.authorBranca, R.
dc.contributor.authorCampilho, F.
dc.contributor.authorRoncon, S.
dc.contributor.authorVasconcelos, C.
dc.contributor.authorCampos, A.
dc.date.accessioned2015-07-13T15:20:59Z
dc.date.available2015-07-13T15:20:59Z
dc.date.issued2014
dc.description.abstractSystemic Sclerosis (SSc) is a chronic disease of the connective tissue, whose pathogenesis involves abnormalities of the immunological system. It has a variable course and there is a subgroup of patients with rapidly progressive disease or unresponsive to conventional treatment. Thesepatients can benefit from intensive immunosuppression and autologous hematopoietic stem cell transplant. Clinical case: 19-year-old (y.o.) woman diagnosed with SSc at the age of 13 y.o. with cutaneous, vascular and articular involvement with initial response to me thotrexate. Three years later the disease progressed with severe digestive involvement (dysphagia, delayed gastric emptying and weight loss) needing gastrostomy for nutritional support. She was treated with cyclophosphamide without improvement. In May 2012 she had an autologous transplant with myeloablative regimen (BEAM): carmustine 300 mg/m2 x1day; etoposide 120 mg/kg x4days; cytarabine 120 mg/kg 12/12:h x4days; melphalan 140 mg/m2 x1day. A year and a half after transplantation she is asymptomatic, without any signs or symptoms of the disease, feeds by mouth and the gastric emptying study is normal. Currently she is free of medication.por
dc.identifier.citationACTA REUMATOL PORT. 2014;39:262-264por
dc.identifier.issn0303-464X
dc.identifier.urihttp://hdl.handle.net/10400.16/1816
dc.language.isoengpor
dc.peerreviewedyespor
dc.publisherSociedade Portuguesa de Reumatologiapor
dc.relation.publisherversionhttp://www.ncbi.nlm.nih.gov/pubmed/?term=PMID%3A+25326407por
dc.subjectSystemic sclerosispor
dc.subjectAutologous Hematopoietic Stem Cell Transplantpor
dc.titleAutologous stem cell transplantation in a patient with severe systemic sclerosispor
dc.typejournal article
dspace.entity.typePublication
oaire.citation.conferencePlacePortugalpor
oaire.citation.endPage264por
oaire.citation.startPage262por
oaire.citation.titleActa Reumatológica Portuguesapor
oaire.citation.volume2014 (39)por
rcaap.rightsopenAccesspor
rcaap.typearticlepor

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