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Systemic AL Amyloidosis

Plasma cell disorder producing amyloidogenic light chains and organ damage

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Hematoloji department. Book Appointment →

What is Systemic AL Amyloidosis?

Systemic AL amyloidosis arises from a small clonal plasma cell population in bone marrow that secretes free monoclonal kappa or lambda light chains that misfold and deposit as fibrils in target organs. Median age at diagnosis is around sixty five with male predominance and many patients have non specific fatigue and weight loss before organ specific manifestations.

Diagnostic workup combines monoclonal protein detection with serum and urine immunofixation, free light chain assay, biopsy of abdominal fat or affected organ stained with Congo red showing apple green birefringence under polarized light, and mass spectrometry typing to confirm AL versus other amyloid forms. Cardiac involvement is assessed by troponin, NT proBNP, echocardiography with strain imaging, and cardiac magnetic resonance.

Treatment aims to suppress the clonal plasma cell rapidly with bortezomib cyclophosphamide dexamethasone plus daratumumab as standard frontline therapy in the Andromeda regimen. Eligible patients with limited cardiac involvement may receive autologous stem cell transplantation. Hematologic response and organ response are tracked at three month intervals; relapse may need lenalidomide based or melphalan based regimens.

Symptoms

Macroglossia and periorbital purpura
Carpal tunnel syndrome bilateral
Heart failure with preserved ejection fraction
Nephrotic range proteinuria
Autonomic and peripheral neuropathy

Risk Factors

Older adult age
Underlying monoclonal gammopathy
Plasma cell smoldering disease
Multiple myeloma related state
Genetic factors limited

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • When unexplained heart failure with low voltage occurs
  • When nephrotic syndrome appears with monoclonal gammopathy
  • When peripheral neuropathy progresses
  • When suspected amyloid biopsy is needed
  • When organ function deteriorates rapidly

Treatment Methods

01
Bortezomib cyclophosphamide dexamethasone daratumumab
02
Autologous stem cell transplantation if eligible
03
Lenalidomide based regimens at relapse
04
Cardiac monitoring and heart failure management
05
Renal protective measures and dialysis if needed
06
Diuretics with caution for hypotension
07
Multidisciplinary supportive care

Which Department to Visit?

You can visit our Hematoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.