Amyloidosis

Terrell Benold, MD Reviewed 06/2017
 


BASICS

DESCRIPTION

  • Very uncommon disorder in which proteins change conformation, aggregate, and form fibrils that infiltrate tissues, leading to organ failure and death. Deposition of amyloid in the ex...

DIAGNOSIS

HISTORY

  • Four essential steps in diagnosing AL:

    • Consider the diagnosis.

    • Make the diagnosis by tissue biopsy.

    • Determine the precursor protein.

    • Define the extent of involvement (2)[A].

  • Careful fami...

TREATMENT

GENERAL MEASURES

  • Familial (ATTR): liver transplant to remove site of synthesis of the mutant protein

  • Hemodialysis and renal transplantation for renal amyloid

  • Hemodialysis-related amyloidosis: C...

ONGOING CARE

FOLLOWUP RECOMMENDATIONS

MONITORING

  • Routine monitoring of renal function

  • Monitor adverse effects of medications.

  • AA: Maintenance of low SAA protein levels correlates to better outcome.

  • AL: Pro...

REFERENCES

Mahmood S, Palladini G, Sanchorawala V et al. Update on treatment of light chain amyloidosis. Haematologica.  2014;99(2):209–221. [View Abstract on OvidInsights]
Dispenzieri A, Gertz ...

ADDITIONAL READING

SEE ALSO

Multiple Myeloma 

CODES

ICD10

  • E85.9 Amyloidosis, unspecified

  • E85.3 Secondary systemic amyloidosis

  • E85.2 Heredofamilial amyloidosis, unspecified

  • E85.8 Other amyloidosis

  • E85.4 Organ-limited amyloidosis

  • E85.1 Neuropathic heredo...

PEARLS

  • The most commonly affected organs resulting in symptoms are heart, kidneys, skin, peripheral and autonomic nerves, and liver; early detection is key to improved survival; respective means of pr...

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