[HTML][HTML] Outcome of patients with immunoglobulin light-chain amyloidosis with lung, liver, gastrointestinal, neurologic, and soft tissue involvement after autologous …

A Afrough, RM Saliba, A Hamdi, R El Fakih… - Biology of Blood and …, 2015 - Elsevier
A Afrough, RM Saliba, A Hamdi, R El Fakih, A Varma, YT Dinh, G Rondon, AM Cornelison…
Biology of Blood and Marrow Transplantation, 2015Elsevier
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 …
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.
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