RENAL AMYLOIDOSIS - AL TYPE
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ALTERNATE NAMES
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Amyloidosis Associated Kidney Disease; Kidney Amyloidosis; Kidney Amyloidosis AL Type;
Renal Amyloidosis; Renal AL Amyloidosis
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DESCRIPTION
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Amyloidosis is a rare disease that occurs when amyloid proteins are deposited in tissues
and organs. Amyloid proteins are abnormal proteins that the body cannot break down
and recycle, as it does with normal proteins. When amyloid proteins clump together,
they form amyloid deposits. The buildup of these deposits damages a person’s organs
and tissues. The kidneys are the organs most commonly affected by primary amyloidosis.
In Renal Amyloidosis, amyloid deposits damage the kidneys and make it harder for them to filter wastes
and break down proteins. When the kidneys become too damaged, they may no longer be
able to function well enough to maintain health, resulting in kidney failure. Kidney
failure can lead to problems such as high blood pressure, bone disease, and anemia.
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9/ICD-10-CM
CODING
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Diagnostic testing: The diagnosis of renal amyloidosis is made by:
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Medical and family history;
Physical findings: The physical findings include:
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Albuminuria (increased amount of albumin, a protein, in the urine);
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Hyperlipidemia (blood has more-than-normal amounts of fats and cholesterol);
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Edema (swelling of the legs, feet, or ankles and less often the hands or face); and
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Hypoalbuminemia (less-than-normal amounts of albumin).
Other signs and symptoms of primary amyloidosis may include:
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Fatigue or feeling tired;
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Numbness, tingling, or a burning sensation in the hands or feet; and
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Unintentional weight loss.
ICD-9: 277.3, 277.39
ICD-10: E85.4
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PROGRESSION
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Amyloidosis that affects the kidneys leads to life-threatening kidney failure and
end-stage renal disease. AL-Amyloidosis has very poor prognosis with most patients
dying in 2-3 years from diagnosis.
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TREATMENT
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AL Amyloidosis is difficult to treat, and treatment is generally only marginally effective.
Treatment may consist of medication therapy, chemotherapy, and stem cell transplant.
Chemotherapy and bone marrow (stem cell) transplant tend to be the most effective
therapies.
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SUGGESTED PROGRAMMATIC ASSESSMENT*
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Suggested MER for Evaluation:
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Clinical history and examination that describes the diagnostic features of renal involvement;
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Suggested Listings for Evaluation:
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DETERMINATION
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LISTING
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REMARKS
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Meets
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Equals
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6.05 A
14.03
14.07 B
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Only the conditions of 6.05 A must be met.
Use listing 14.07 B when a stem cell or bone marrow transplant has been completed.
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* Adjudicators may, at their discretion, use the Medical Evidence of Record or the
listings suggested evaluating the claim. However, the decision to allow or deny the
claim rests with the adjudicator.
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