TY - JOUR
T1 - Outcome of Patients with Immunoglobulin Light-Chain Amyloidosis with Lung, Liver, Gastrointestinal, Neurologic, andSoft Tissue Involvement after Autologous Hematopoietic Stem Cell Transplantation
AU - Afrough, Aimaz
AU - Saliba, Rima M.
AU - Hamdi, Amir
AU - El Fakih, Riad
AU - Varma, Ankur
AU - Dinh, Yvonne T.
AU - Rondon, Gabriela
AU - Cornelison, A. Megan
AU - Shah, Nina D.
AU - Bashir, Qaiser
AU - Shah, Jatin J.
AU - Hosing, Chitra
AU - Popat, Uday
AU - Orlowski, Robert Z.
AU - Champlin, Richard E.
AU - Parmar, Simrit
AU - Qazilbash, Muzaffar H.
N1 - Publisher Copyright:
© 2015 American Society for Blood and Marrow Transplantation.
PY - 2015/8/1
Y1 - 2015/8/1
N2 - There is limited information on the outcome when organs other than heart or kidneys are involved by immunoglobulin light-chain amyloidosis (AL). We report the outcome of 53 patients with AL with gastrointestinal (GI), peripheral nerve (PN), liver, lung, or soft-tissue involvement, who underwent high-dose chemotherapy and autologous hematopoietic stem cell transplantation (auto-HCT) at our institution between 1997 and 2013. The median age at auto-HCT was 56 years (range, 35 to 74). One, 2, 3, or 4 organs were involved in 43%, 22%, 28%, and 4% of patients, respectively. Concurrent cardiac, renal, or both were involved in 24 (45%) patients. Forty-six patients received induction therapy before auto-HCT. The 100-day and 1-year treatment-related mortality (TRM) were 3.8% (n= 2) and 7.5% (n= 4), respectively. Forty-one (80%) patients achieved a hematologic response. Organ response at 1 year after auto-HCT was seen in 23 (57%) of the 40 evaluable patients. With a median follow-up of 24 months, the median progression-free survival and overall survival (OS) were 36 and 73 months, respectively. Auto-HCT was associated with a low TRM, durable organ responses, and a median OS of > 6 years in selected patients with AL and GI, PN, liver, lung, or softtissue involvement.
AB - There is limited information on the outcome when organs other than heart or kidneys are involved by immunoglobulin light-chain amyloidosis (AL). We report the outcome of 53 patients with AL with gastrointestinal (GI), peripheral nerve (PN), liver, lung, or soft-tissue involvement, who underwent high-dose chemotherapy and autologous hematopoietic stem cell transplantation (auto-HCT) at our institution between 1997 and 2013. The median age at auto-HCT was 56 years (range, 35 to 74). One, 2, 3, or 4 organs were involved in 43%, 22%, 28%, and 4% of patients, respectively. Concurrent cardiac, renal, or both were involved in 24 (45%) patients. Forty-six patients received induction therapy before auto-HCT. The 100-day and 1-year treatment-related mortality (TRM) were 3.8% (n= 2) and 7.5% (n= 4), respectively. Forty-one (80%) patients achieved a hematologic response. Organ response at 1 year after auto-HCT was seen in 23 (57%) of the 40 evaluable patients. With a median follow-up of 24 months, the median progression-free survival and overall survival (OS) were 36 and 73 months, respectively. Auto-HCT was associated with a low TRM, durable organ responses, and a median OS of > 6 years in selected patients with AL and GI, PN, liver, lung, or softtissue involvement.
KW - Amyloidosis
KW - Autologous hematopoietic stem cell transplantation
KW - Response
KW - Survival
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U2 - 10.1016/j.bbmt.2015.03.025
DO - 10.1016/j.bbmt.2015.03.025
M3 - Article
C2 - 25842049
AN - SCOPUS:84937732098
SN - 1083-8791
VL - 21
SP - 1413
EP - 1417
JO - Biology of Blood and Marrow Transplantation
JF - Biology of Blood and Marrow Transplantation
IS - 8
ER -