TY - JOUR
T1 - Coagulation Abnormalities in Light Chain Amyloidosis
AU - Abdallah, Nadine
AU - Muchtar, Eli
AU - Dispenzieri, Angela
AU - Gonsalves, Wilson
AU - Buadi, Francis
AU - Lacy, Martha Q.
AU - Hayman, Suzanne R.
AU - Kourelis, Taxiarchis
AU - Kapoor, Prashant
AU - Go, Ronald S.
AU - Warsame, Rahma
AU - Leung, Nelson
AU - Rajkumar, S. Vincent
AU - Kyle, Robert A.
AU - Pruthi, Rajiv K.
AU - Gertz, Morie A.
AU - Kumar, Shaji K.
N1 - Funding Information:
Potential Competing Interests: Dr Kapoor received research funding from Takeda Pharmaceuticals , Celgene , and Amgen . Dr Dispenzieri received research funding from Celgene , Millennium Pharmaceuticals , Pfizer , and Janssen , and received a travel grant from Pfizer . Dr Gertz served as a consultant for Millennium Pharmaceuticals and received honoraria from Celgene , Millennium Pharmaceuticals , Onyx Pharmaceuticals , Novartis , GlaxoSmithKline , Prothena , Ionis Pharmaceuticals , and Amgen . Dr Lacy received research funding from Celgene . Dr Leung serves on an advisory board for Takeda Pharmaceuticals. Dr Kumar served as a consultant for Celgene, Millennium Pharmaceuticals, Onyx Pharmaceuticals, Janssen, and Bristol-Myers Squibb, and received research funding from Celgene , Millennium Pharmaceuticals , Novartis , Onyx Pharmaceuticals , AbbVie , Janssen , and Bristol-Myers Squibb . The remaining authors declare no competing financial interests.
Funding Information:
Potential Competing Interests: Dr Kapoor received research funding from Takeda Pharmaceuticals, Celgene, and Amgen. Dr Dispenzieri received research funding from Celgene, Millennium Pharmaceuticals, Pfizer, and Janssen, and received a travel grant from Pfizer. Dr Gertz served as a consultant for Millennium Pharmaceuticals and received honoraria from Celgene, Millennium Pharmaceuticals, Onyx Pharmaceuticals, Novartis, GlaxoSmithKline, Prothena, Ionis Pharmaceuticals, and Amgen. Dr Lacy received research funding from Celgene. Dr Leung serves on an advisory board for Takeda Pharmaceuticals. Dr Kumar served as a consultant for Celgene, Millennium Pharmaceuticals, Onyx Pharmaceuticals, Janssen, and Bristol-Myers Squibb, and received research funding from Celgene, Millennium Pharmaceuticals, Novartis, Onyx Pharmaceuticals, AbbVie, Janssen, and Bristol-Myers Squibb. The remaining authors declare no competing financial interests.
Publisher Copyright:
© 2020 Mayo Foundation for Medical Education and Research
PY - 2021/2
Y1 - 2021/2
N2 - Objective: To assess the prevalence of coagulation abnormalities in patients with systemic light chain (AL) amyloidosis and their association with disease-related characteristics, disease progression, and survival. Patients and Methods: This is a retrospective study of patients with AL amyloidosis seen at Mayo Clinic, Rochester, Minnesota, from January 1, 2006, to December 31, 2015. We studied the association between abnormal coagulation parameters and baseline characteristics and their association with survival outcomes. Results: The study included 411 patients. Abnormalities at diagnosis included prolonged clotting times and coagulation factor deficiencies; prolonged prothrombin time (PT) and factor X (FX) deficiency were found in 19% (73 of 390) and 43% (177 of 411) of patients, respectively. The FX deficiency was associated with higher Mayo stage, involvement of more than 1 organ, liver and cardiac involvement, and greater than 10% bone marrow plasma cells. On univariate analysis, the risk for disease progression or death was higher in patients with abnormal values for PT and factor V, factor VII (FVII), FX, and factor XII compared with those with normal values. Prolonged PT and FVII and FX deficiencies were independent predictors of death after adjusting for Mayo stage and more than 1 organ involvement. Only 106 patients had repeat testing after treatment; no clear relationship was found between treatment response and changes in coagulation parameters. Conclusion: Coagulation abnormalities occur in a significant proportion of patients with AL amyloidosis and are associated with advanced disease and inferior outcomes. Larger studies are needed to establish whether a relationship exists between treatment response and improvement in individual parameters.
AB - Objective: To assess the prevalence of coagulation abnormalities in patients with systemic light chain (AL) amyloidosis and their association with disease-related characteristics, disease progression, and survival. Patients and Methods: This is a retrospective study of patients with AL amyloidosis seen at Mayo Clinic, Rochester, Minnesota, from January 1, 2006, to December 31, 2015. We studied the association between abnormal coagulation parameters and baseline characteristics and their association with survival outcomes. Results: The study included 411 patients. Abnormalities at diagnosis included prolonged clotting times and coagulation factor deficiencies; prolonged prothrombin time (PT) and factor X (FX) deficiency were found in 19% (73 of 390) and 43% (177 of 411) of patients, respectively. The FX deficiency was associated with higher Mayo stage, involvement of more than 1 organ, liver and cardiac involvement, and greater than 10% bone marrow plasma cells. On univariate analysis, the risk for disease progression or death was higher in patients with abnormal values for PT and factor V, factor VII (FVII), FX, and factor XII compared with those with normal values. Prolonged PT and FVII and FX deficiencies were independent predictors of death after adjusting for Mayo stage and more than 1 organ involvement. Only 106 patients had repeat testing after treatment; no clear relationship was found between treatment response and changes in coagulation parameters. Conclusion: Coagulation abnormalities occur in a significant proportion of patients with AL amyloidosis and are associated with advanced disease and inferior outcomes. Larger studies are needed to establish whether a relationship exists between treatment response and improvement in individual parameters.
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U2 - 10.1016/j.mayocp.2020.06.061
DO - 10.1016/j.mayocp.2020.06.061
M3 - Article
C2 - 33549257
AN - SCOPUS:85100435678
SN - 0025-6196
VL - 96
SP - 377
EP - 387
JO - Mayo Clinic proceedings
JF - Mayo Clinic proceedings
IS - 2
ER -