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AL amyloidosis
See also in: External and Internal Eye,Oral Mucosal Lesion
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AL amyloidosis

See also in: External and Internal Eye,Oral Mucosal Lesion
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Contributors: Noah Craft MD, PhD, Lindy P. Fox MD, Lowell A. Goldsmith MD, MPH, Michael D. Tharp MD
Other Resources UpToDate PubMed

Synopsis

Primary systemic (AL) amyloidosis is an acquired amyloidosis, almost always associated with an immunocyte dyscrasia. Multiple myeloma is the most common association, but it is also seen with Waldenstrom macroglobulinemia and other paraproteinemias. The disease is more common in older adults. The prognosis is poor.

Amyloid deposits in primary systemic amyloidosis are composed of immunoglobulin light chains. Monoclonal proteins or Bence Jones proteins are usually detected. Neurologic symptoms include a sensory peripheral neuropathy, presenting in a stocking and glove distribution. An "idiopathic" carpal tunnel syndrome can also occur.

Systemic findings can be related to the involvement of the heart, muscles, gastrointestinal tract, kidneys, and nerves. Cardiac symptoms and low ECG voltages may develop as a consequence of restrictive cardiomyopathy. Renal involvement with nephrotic syndrome is the most common presentation of immunoglobulin amyloidosis.

Primary systemic amyloidosis may present with skin and/or mucosal lesions before systemic involvement is apparent, providing an early clue to diagnosis.

The incidence of this disease increases with advancing age.

Related topic: AA amyloidosis

Codes

ICD10CM:
E85.9 – Amyloidosis, unspecified

SNOMEDCT:
89449005 – Systemic amyloidosis

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Differential Diagnosis & Pitfalls

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Therapy

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References

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Last Updated: 07/17/2018
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AL amyloidosis
See also in: External and Internal Eye,Oral Mucosal Lesion
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AL amyloidosis : Hepatomegaly, Alkaline phosphatase elevated, Eyelid edema, Eyelids, Neuropathy peripheral, Proteinuria, Tongue, Tongue edema, Ecchymosis, Splenomegaly
Clinical image of AL amyloidosis
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