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Serum free light chain trends between orthotopic heart transplantation and auto-SCT in patients with AL amyloidosis

Bone Marrow Transplantation volume 50, pages 868–869 (2015)Cite this article

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Cardiac involvement, present in about 50% of patients, is the leading cause of morbidity and mortality in immunoglobulin light chain (AL) amyloidosis. Cardiac biomarkers can risk stratify the patients, and those with advanced (stage III)1 disease, who have the poorest prognosis, regardless of treatment, with a median survival of 3.5–7 months.2 High-dose melphalan and auto-SCT (HDM/SCT), which produces a high rate of hematologic responses,3 can be used in highly selected patients with stage III disease,4 but for most stage III patients, treatment-related mortality is high. The patients with advanced cardiac involvement and preserved function of other organs have undergone orthotopic heart transplantation (OHT), but OHT alone leaves patients susceptible to rapid recurrence of AL amyloidosis in the transplanted heart and other organs.5 We have found that although mortality on the waiting list is high for AL patients, if OHT is carried out successfully and is followed by HDM/SCT, survival is similar to that of patients undergoing OHT for other indications.6, 7 Comparable data have been reported from the Mayo Clinic8 and the UK National Amyloidosis Centre.9 The need for antiplasma cell therapy while awaiting HDM/SCT has not been demonstrated, although in a case series of three patients, lenalidomide or bortezomib-based chemotherapy has been employed.10 Here we evaluate the changes in serum free light chain (FLC) levels in the interval between planned OHT and HDM/SCT, to help guide the need for treatment.

Seventeen patients were treated following collaborative protocols at the Boston Medical Center Amyloidosis Center and the Massachusetts General Hospital Heart Failure Program, between 1997 and 2013. Data were reviewed with approval of both hospitals’ Institutional Review Boards. All patients had a diagnosis of AL amyloidosis; patients with symptomatic multiple myeloma (BM plasma cell percentage >30% or hypercalcemia, anemia or lytic bone disease) were not considered OHT candidates.

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References

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Authors and Affiliations

  1. Amyloidosis Center, Boston University School of Medicine and Boston Medical Center, Boston, MA, USA

    A S Renteria, V Sanchorawala & D C Seldin

  2. Heart Failure and Cardiac Transplant Program, Harvard Medical School, Massachusetts General Hospital, Boston, MA, USA

    E D Niehaus & M J Semigran

  3. Department of Biostatistics, Boston University School of Public Health, Boston, MA, USA

    F Sun

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  1. A S Renteria
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  2. V Sanchorawala
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Correspondence to D C Seldin.

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Renteria, A., Sanchorawala, V., Niehaus, E. et al. Serum free light chain trends between orthotopic heart transplantation and auto-SCT in patients with AL amyloidosis. Bone Marrow Transplant 50, 868–869 (2015). https://doi.org/10.1038/bmt.2015.9

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