scholarly journals Autologous hematopoietic stem cell transplantation in light chain amyloidosis (AL) with renal involvement

2015 ◽  
Vol 51 (2) ◽  
pp. 307-309 ◽  
Author(s):  
A M Cornelison ◽  
R M Saliba ◽  
A Afrough ◽  
Y Dinh ◽  
Y Nieto ◽  
...  
Keyword(s):  
Stem Cell ◽  
Hematopoietic Stem Cell Transplantation ◽  
Stem Cell Transplantation ◽  
Cell Transplantation ◽  
Hematopoietic Stem Cell ◽  
Light Chain ◽  
Renal Involvement ◽  
Hematopoietic Stem ◽  
Light Chain Amyloidosis

Progressive Renal Light Chain Amyloidosis With the Absence of Detectable Free Monoclonal Light Chains After an Autologous Hematopoietic Stem Cell Transplant for Amyloid Light Chain Amyloidosis

2013 ◽  
Vol 137 (9) ◽  
pp. 1304-1308 ◽  
Author(s):  
Rachel M. Roth ◽  
Don Benson ◽  
Lee A. Hebert ◽  
Michael G. Bissell ◽  
Anjali A. Satoskar ◽  
...  
Keyword(s):  
Stem Cell ◽  
Hematopoietic Stem Cell Transplantation ◽  
Stem Cell Transplantation ◽  
Cell Transplantation ◽  
Hematopoietic Stem Cell ◽  
Light Chain ◽  
Hematopoietic Stem ◽  
Monoclonal Protein ◽  
Light Chain Amyloidosis ◽  
Amyloid Light Chain

Amyloid light chain amyloidosis involving the kidneys is not uncommon in patients with monoclonal gammopathy. The mainstay of treatment of amyloid light chain amyloidosis is autologous hematopoietic stem cell transplantation. Evidence that the autologous hematopoietic stem cell transplantation has been successful is the absence of free monoclonal light chains in serum and urine. Herein, we report 2 cases of progressive renal amyloid light chain amyloidosis after autologous hematopoietic stem cell transplantation, documented by kidney biopsy, despite the absence of monoclonal protein in the serum and urine. Kidney function declined progressively in both patients. During that time, numerous immunofixation and protein electrophoresis test results were negative for monoclonal protein, both in serum and urine, concealing the progression of the amyloidosis. We conclude that close monitoring of kidney function is warranted following autologous hematopoietic stem cell transplantation in patients with amyloid light chain amyloidosis, even with negative results from monoclonal protein testing. Unexplained, worsening renal function warrants a kidney biopsy to assess whether retreatment of the monoclonal gammopathy is indicated.

scholarly journals High serum neurofilament light chain normalizes after hematopoietic stem cell transplantation for MS

2019 ◽  
Vol 6 (5) ◽  
pp. e598 ◽  
Author(s):  
Simon Thebault ◽  
Daniel R. Tessier ◽  
Hyunwoo Lee ◽  
Marjorie Bowman ◽  
Amit Bar-Or ◽  
...  
Keyword(s):  
Stem Cell ◽  
Hematopoietic Stem Cell Transplantation ◽  
Stem Cell Transplantation ◽  
Disease Severity ◽  
Cell Transplantation ◽  
Hematopoietic Stem Cell ◽  
Light Chain ◽  
Neurofilament Light Chain ◽  
Neurofilament Light ◽  
Hematopoietic Stem

ObjectiveTo evaluate neurofilament light chain (NfL) levels in serum and CSF of patients with aggressive MS pre- and post-treatment with immunoablation followed by autologous hematopoietic stem cell transplantation (IAHSCT) and examine associations with clinical and MRI outcomes.MethodsPaired serum and CSF in addition to MRI and clinical measures were collected on 23 patients with MS at baseline and 1 and 3 years post-IAHSCT. An additional 33 sera and CSF pairs were taken from noninflammatory neurologic controls. NfL levels were quantitated using the Simoa platform (Quanterix).ResultsBaseline MS NfL levels were significantly elevated relative to controls in serum (p = 0.001) and CSF (p = 0.001). Following IAHSCT, high pretreatment NfL levels significantly reduced in serum (p = 0.0023) and CSF (p = 0.0068) and were not significantly different from controls. Serum and CSF NfL levels highly correlated (r = 0.81, p < 0.0001). Baseline NfL levels were associated with worse pretreatment disease measures (Expanded Disability Status Scale [EDSS], relapses, MRI lesions, and MR spectroscopy (MRS) N-acetylaspartate/creatine). Elevated baseline NfL levels were associated with persistently worse indices of disease burden post-IAHSCT (sustained EDSS progression, cognition, quality of life, T1 and T2 lesion volumes, MRS, and brain atrophy).ConclusionThese data substantiate that serum and CSF NfL levels reflect disease severity and treatment response in patients with MS and may therefore be a useful biomarker. Baseline serum levels associated with markers of pretreatment disease severity and post-treatment outcomes.Classification of evidenceThis study provides Class II evidence that for patients with aggressive MS, serum NfL levels are associated with disease severity.

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