Outcome of Patients with Immunoglobulin Light-Chain Amyloidosis with Lung, Liver, Gastrointestinal, Neurologic, and Soft Tissue Involvement after Autologous Hematopoietic Stem Cell Transplantation.

Abstract

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 soft-tissue involvement.

• Authors: Aimaz Afrough, Rima M Saliba, Amir Hamdi, Riad El Fakih, Ankur Varma, Yvonne T Dinh, Gabriela Rondon, A Megan Cornelison, Nina D Shah, Qaiser Bashir, Jatin J Shah, Chitra Hosing, Uday Popat, Robert Z Orlowski, Richard E Champlin, Simrit Parmar, Muzaffar H Qazilbash
• Journal: Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation (Biol Blood Marrow Transplant) Vol. 21 Issue 8 Pg. 1413-7 (Aug 2015) ISSN: 1523-6536 [Electronic] United States
• PMID: 25842049 (Publication Type: Journal Article)
• Copyright: Copyright © 2015 American Society for Blood and Marrow Transplantation. Published by Elsevier Inc. All rights reserved.

Chemical References

• Immunoglobulin Light Chains

Topics

• Adult
• Aged
• Amyloidosis (complications, mortality, therapy)
• Female
• Hematopoietic Stem Cell Transplantation (methods)
• Humans
• Immunoglobulin Light Chains (metabolism)
• Male
• Middle Aged
• Survival Rate
• Transplantation Conditioning (methods)
• Transplantation, Autologous (methods)
• Treatment Outcome