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تعداد آیتم قابل مشاهده باقیمانده : 3 مورد

Immunoglobulin light chain (AL) amyloidosis response assessment

Immunoglobulin light chain (AL) amyloidosis response assessment
System Response
Hematologic[1,2]

CR: Negative serum and urine immunofixation plus either normalization of the FLC ratio or uninvolved FLC concentration greater than involved FLC concentration with or without an abnormal FLC ratio

VGPR: Reduction in the dFLC to <40 mg/L*

PR: A >50% reduction in the dFLC*

No response: Less than a PR

Cardiac[3]

CR: Nadir NT-proBNP ≤350 pg/mL (≤41.39 pmol/L) or BNP ≤80 pg/mL (≤9.46 pmol/L)

VGPR: >60% reduction in NT-proBNP/BNP from baseline level, not meeting cardiac CR criteria

PR: 31 to 60% reduction in NT-proBNP from baseline level not meeting CR

No response: ≤30% reduction in NT-proBNP from baseline level

Kidney[4]

CR: Nadir proteinuria ≤200 mg/24 h

VGPR: >60% reduction in proteinuria from baseline level, not meeting renal CR criteria

PR: 31 to 50% reduction in proteinuria from baseline level, not meeting renal CR criteria

No response: ≤30% reduction in proteinuria from baseline level

Liver[4]

CR: Nadir alkaline phosphatase ≤2 times lower limit of normal

VGPR: >60% reduction in alkaline phosphatase from baseline level, not meeting hepatic CR criteria

PR: 31 to 50% reduction in alkaline phosphatase from baseline level, not meeting hepatic CR criteria

No response: <50% decrease in serum alkaline phosphatase from baseline value

BNP: B-type natriuretic peptide; CR: complete response; dFLC: difference between involved FLC and uninvolved FLC; FLC: free light chain; NT-proBNP: N-terminal pro-B-type natriuretic peptide; PR: partial response; VGPR: very good partial response.

* The dFLC values used in these response criteria do not apply to the approximately 20% of patients with a pretreatment dFLC <50 mg/L. If the pretreatment dFLC is between 20 and 50 mg/L, a dFLC <10 mg/L can be used to define a dFLC-based PR[5,6].

References:
  1. Palladini G, Dispenzieri A, Gertz MA, et al. New criteria for response to treatment in immunoglobulin light chain amyloidosis based on free light chain measurement and cardiac biomarkers: impact on survival outcomes. J Clin Oncol 2012; 30:4541.
  2. Palladini G, Schönland SO, Sanchorawala V, et al. Clarification on the definition of complete haematologic response in light-chain (AL) amyloidosis. Amyloid 2021; 28:1.
  3. Muchtar E, Dispenzieri A, Wisniowski B, et al. Graded cardiac response criteria for patients with systemic light chain amyloidosis. J Clin Oncol 2023; 41:1393.
  4. Muchtar E, Dispenzieri A, Leung N, et al. Depth of organ response in AL amyloidosis is associated with improved survival: grading the organ response criteria. Leukemia 2018; 32:2240.
  5. Milani P, Basset M, Russo F, et al. Patients with light-chain amyloidosis and low free light-chain burden have distinct clinical features and outcome. Blood 2017; 130:625.
  6. Dittrich T, Bochtler T, Kimmich C, et al. AL amyloidosis patients with low amyloidogenic free light chain levels at first diagnosis have an excellent prognosis. Blood 2017; 130:632.
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