[en] A patient with Jo-1 antibody-associated polymyositis (Jo-1 PM) had a Karnofsky score of 40% and severe muscle, liver and lung damage that was refractory to standard therapy. The female patient received an autologous T-cell-depleted haematopoietic stem cell transplant (HSCT) after myeloablative conditioning. The transplant procedure was complicated by severe adult respiratory distress syndrome (ARDS) and adenovirus-associated haemorrhagic cystitis as well as cytomegalovirus (CMV) reactivation. The patient's creatinine phosphokinase (CPK) and alanine transaminase (ALT) values were normal on day 21. The patient's strength has improved remarkably and her dyspnoea is subjectively improved. At 15 months after the transplant, the patient was well with a Karnofsky score of 80% and had been off any therapy, including steroids, for 14 months.
Disciplines :
Hematology Rheumatology
Author, co-author :
Baron, Frédéric ; Centre Hospitalier Universitaire de Liège - CHU > Hématologie clinique
Ribbens, Clio ; Centre Hospitalier Universitaire de Liège - CHU > Rhumatologie
Kaye, O.
Fillet, Georges ; Centre Hospitalier Universitaire de Liège - CHU > Hématologie clinique
Malaise, Michel ; Université de Liège - ULiège > Département des sciences cliniques > Rhumatologie
Beguin, Yves ; Centre Hospitalier Universitaire de Liège - CHU > Hématologie clinique
Language :
English
Title :
Effective treatment of Jo-1-associated polymyositis with T-cell-depleted autologous peripheral blood stem cell transplantation.
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