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Secondary systemic amyloidosis

Definition

Secondary systemic amyloidosis is a disorder in which abnormal proteins build up in tissues and organs. Clumps of the abnormal proteins are called amyloid deposits.

Secondary means it occurs because of another disease or situation. For example, this condition usually occurs as a result of chronic infection or chronic inflammatory disease. Primary amyloidosis means there is no disease that is causing the condition.

Systemic means body-wide. For example, a systemic disease affects the entire body.

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Alternative Names

Amyloidosis - secondary systemic

Causes, incidence, and risk factors

The exact cause of amyloidosis is unknown. You are more likely to develop secondary systemic amyloidosis if you have a long-term infection or inflammation.

This condition may occur with:

Symptoms

Bleeding in the skin
Fatigue
Irregular heartbeat
Numbness of hands and feet
Rash
Shortness of breath
Swallowing difficulties
Swollen arms or legs
Swollen tongue
Weak hand grip
Weight loss

Signs and tests

Tests that may be done include:

Abdominal ultrasound (may show a swollen liver or spleen)
Biopsy or aspiration of fat just beneath the skin (subcutaneous fat)
Biopsy of rectum
Biopsy of skin
Biopsy of bone marrow
Blood tests, including creatinine and BUN
Echocardiogram
Electrocardiogram (ECG)
Nerve conduction velocity
Urinalysis

Treatment

The condition that is causing the amyloidosis should be treated. Some persons may need a bone marrow or stem cell transplant. There is no specific treatment for amyloidosis itself.

Expectations (prognosis)

How well a person does depends on which organs are affected and whether the disease that is causing it can be controlled. If the disease involves the heart and kidneys, it may lead to organ failure and death.

Complications

Calling your health care provider

Call your health care provider if you have symptoms of this condition. The following are serious symptoms that need prompt medical attention:

Bleeding
Irregular heartbeat
Numbness
Shortness of breath
Swelling
Weak grip

Prevention

If you have a disease that's known to increase your risk of this condition, make sure you get it treated. This may help prevent amyloidosis.

References

Buxbaum JN. The amyloidoses. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 296.

Gertz MA, Lacy MQ, Dispenzieri A, Hayman SR. Immunologic light-chain amyloidosis (primary amyloidosis). In: Hoffman R, Benz EJ Jr., Shattil SJ, et al, eds. Hoffman Hematology: Basic Principles and Practice. 5th ed. Philadelphia, Pa: Churchill Livingstone Elsevier; 2008:chap 89.

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