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AL Amyloidosis (Immunoglobulin Light Chain Amyloidosis)

Systemic disease formed by deposition of light chain proteins in organs.

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 Dahiliye (İç Hastalıkları) department. Book Appointment →

What is AL Amyloidosis (Immunoglobulin Light Chain Amyloidosis)?

AL amyloidosis is a systemic disease that arises from deposition of abnormal immunoglobulin light chain proteins produced by clonal plasma cells in various organs in the form of amyloid fibrils. It is seen in 8-12 cases per million per year.

The most commonly affected organs are heart, kidney, liver, nervous system, and soft tissues. Cardiac involvement is the most important prognostic factor; restrictive cardiomyopathy and heart failure may develop.

For diagnosis, tissue biopsy with Congo red staining and demonstration of green birefringence under polarized light is required. Mass spectrometry is used for amyloid typing. Treatment aims at suppression of the underlying clonal disease.

Symptoms

Nephrotic-range proteinuria and renal failure
Heart failure findings (shortness of breath, edema)
Peripheral neuropathy and autonomic dysfunction
Macroglossia (enlarged tongue)
Periorbital purpura (bruising around the eyes)
Hepatomegaly
Carpal tunnel syndrome

Risk Factors

History of monoclonal gammopathy (MGUS)
Multiple myeloma
Waldenström macroglobulinemia
Age over 60
Male gender (slight predominance)

When to See a Doctor?

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

  • Combination of unexplained nephrotic syndrome and heart failure
  • Periorbital purpura and macroglossia
  • Detection of monoclonal protein in serum or urine with organ damage
  • Peripheral neuropathy and autonomic dysfunction

Treatment Methods

01
Chemotherapy (bortezomib-based regimens: VCD, CyBorD)
02
Daratumumab + bortezomib combination (new standard)
03
High-dose melphalan with autologous stem cell transplantation (in eligible patients)
04
Organ support therapies (diuretic, ACE inhibitor)
05
Device therapy and transplantation evaluation in cardiac involvement
06
Regular monitoring of free light chain response

Which Department to Visit?

You can visit our Dahiliye (İç Hastalıkları) 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.