title: [Long-term survival after autologous peripheral blood stem cell transplantation in a patient with primary AL amyloidosis complicating congestive heart failure].
abstract: A 44-year-old man was admitted to hospital because of respiratory distress and progressive edema in the lower extremities. He was diagnosed as having congestive heart failure, but his condition improved following intensive care. Echocardiogram revealed a thickened interventricular septum, insufficient diastolic function, and granular sparkling pattern in the ventricular wall. Pathological examination of a myocardial biopsy specimen showed the deposition of AL amyloid, resulting in a diagnosis of AL amyloidosis. He was then referred to our hospital for treatment. After a course of high-dose dexamethasone therapy, peripheral blood stem cells induced by the administration of granulocyte colony stimulating factor were harvested. He then received high-dose melphalan (HDM) with autologous peripheral blood stem cell transplantation (auto-PBSCT) support, leading to complete remission. He has been well for more than three years after the transplantation and enjoys the same daily life as before the onset of symptoms. HDM/auto-PBSCT for AL amyloidosis confers a higher response rate and longer survival than conventional chemotherapies; however, treatment-related toxicity is also high. Refinements of treatment strategies are urgently needed. This case provides insights into appropriate strategies for HDM/auto-PBSCT for AL amyloidosis with regard to patient selection, the best induction therapy, and the risk-adjusted melphalan conditioning dose; all of which should be confirmed by randomized controlled trials.
authors: First Department of Internal Medicine, Sapporo Medical University School of Medicine.
authors: Mayuko
authors: M
authors: Saito
authors: Toshiaki
authors: T
authors: Hayashi
authors: Masahiro
authors: M
authors: Nojima
authors: Hiroshi
authors: H
authors: Ikeda
authors: Isao
authors: I
authors: Tarasawa
authors: Hiroshi
authors: H
authors: Yasui
authors: Tadao
authors: T
authors: Ishida
authors: Masaaki
authors: M
authors: Adachi
authors: Kohzoh
authors: K
authors: Imai
authors: Yasuhisa
authors: Y
authors: Shinomura
mesh_terms: D000328:Adult; D000686:Amyloidosis / Q000150:complications / Q000628:therapy*; D006333:Heart Failure / Q000150:complications*; D006801:Humans; D008297:Male; D036102:Peripheral Blood Stem Cell Transplantation; D012074:Remission Induction; D014182:Transplantation, Autologous