A real-life cohort study of immunoglobulin light-chain (AL) amyloidosis patients ineligible for autologous stem cell transplantation due to severe cardiac involvement or advanced disease

Brunger, A. F.; Nienhuis, Hans L A; Bijzet, Johan; Roeloffzen, Wilfried; Vellenga, Edo; Hazenberg, Bouke P. C.

doi:10.1080/13506129.2020.1714581

Cited by 4 publications

(1 citation statement)

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“…Early diagnosis of cardiac amyloidosis can lead to prolonged survival (33). Despite new treatment strategies, early death rate of AL cardiac amyloidosis still remains very high (34).…”

Section: Prognosis and Treatmentmentioning

confidence: 99%

Light-Chain Cardiac Amyloidosis: A Case Report and Review of the Literature

Gong¹,

Li²,

Dai³

et al. 2021

Preprint

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Background: Light-chain (AL) cardiac amyloidosis is a rare cardiomyopathy associated with poor prognosis. Due to the advances of imaging technology, cardiac amyloidosis can be now sufficiently diagnosed without myocardial biopsy. Case presentation: This study reports a 69-year-old female who was admitted with chest distress, abdominal distension, anorexia, resistant hypotension, oliguria, and hyponatremia. The patient was confirmed as AL cardiac amyloidosis with the aid of cardiac MR 2 years after symptom onset. The patient received palliative care due to the end stage of the disease. Conclusions: Early diagnosis by cardiac imaging and etiology-oriented treatment are of importance to improve prognosis of AL cardiac amyloidosis.

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“…Early diagnosis of cardiac amyloidosis can lead to prolonged survival (33). Despite new treatment strategies, early death rate of AL cardiac amyloidosis still remains very high (34).…”

Section: Prognosis and Treatmentmentioning

confidence: 99%

Light-Chain Cardiac Amyloidosis: A Case Report and Review of the Literature

Gong¹,

Li²,

Dai³

et al. 2021

Preprint

View full text Add to dashboard Cite

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Diagnostic performance of liver stiffness as marker of liver involvement in systemic immunoglobulin light chain (AL) amyloidosis

Brunger,

Tingen,

Bijzet

et al. 2024

Ann Hematol

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Objective: To investigate the diagnostic performance of liver stiffness for detecting liver involvement in immunoglobulin light chain (AL) amyloidosis. Methods: Liver stiffness was measured using transient elastography in 71 patients with systemic AL amyloidosis and 18 patients with wild type transthyretin (ATTRwt) amyloidosis with cardiomyopathy. Both non-invasive consensus criteria and serum amyloid P component (SAP) scintigraphy were used as substitute standards instead of liver biopsy for establishing liver involvement. Results: Liver stiffness was higher in AL amyloidosis patients with liver involvement than in those without: this was observed using both consensus criteria (median 14.4 kPa vs. 8.1 kPa; p = 0.001) and SAP scintigraphy (median 20.9 kPa vs. 6.2 kPa; p < 0.001). Liver stiffness was also higher in AL amyloidosis patients with liver involvement compared to AL and ATTRwt amyloidosis patients with cardiac involvement. Based on receiver operating characteristic (ROC) curves a cut-off value of 14.4 kPa for stiffness was optimal to indicate liver involvement, providing sensitivity and specificity of 50% and 74%, respectively, using the consensus criteria and 63% and 90%, respectively, using SAP scintigraphy as standard. Conclusion: Liver stiffness is a promising tool to establish liver involvement in AL amyloidosis having potential to become part of updated criteria for liver involvement.

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