7717 - AL Amyloidosis (primary amyloidosis)
DBQ: Link to Index of DBQ/Exams by Disability for DC 7717
Definition
A rare bone marrow disorder where plasma cells produce abnormal antibody proteins (immunoglobulins) that are deposited in and around tissues, nerves, and organs.
Etiology
AL amyloidosis (immunoglobulin light chain amyloidosis) is the most common type and can affect the heart, stomach, large intestine, kidneys, skin, nerves and liver. Previously known as primary amyloidosis, AL amyloidosis occurs when the bone marrow produces abnormal antibodies that cannot be broken down. The antibodies are deposited in the tissues as amyloid, interfering with normal function.
Signs & Symptoms
Symptoms depend on which organs are affected. They may include:
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swelling (edema)
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fatigue and weakness
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shortness of breath
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chest pain
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numbness and tingling
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bruising or bleeding easily
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pain in the hands or feet
Tests
Laboratory tests of the blood, urine, thyroid, and liver may be analyzed for abnormal proteins. A biopsy may be taken from abdominal fat, bone marrow, or other organs. Also, imaging tests of the organs affected (such as echocardiogram for heart or ultrasound of the liver or spleen) can evaluate the extent of the amyloidosis.
Treatment
AL amyloidosis is considered incurable and progressive. Treatment may include chemotherapy, autologous blood stem cell transplant (ASCT) and organ transplants.
Residuals
During chemotherapy treatment, the person may have complications that include:
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anorexia
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nausea
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vomiting
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mouth ulcers
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alopecia
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fatigue
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decreased number of hematopoietic cells in the bone marrow
Counseling may be needed for emotional support. Following organ transplants, postoperative compliance by the patient with all medications is extremely critical. Noncompliance can trigger rejection, even if the transplanted organ has been functioning well for years.
Special Considerations
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Consider service connection on a presumptive basis as a condition associated with herbicide exposure (38 CFR 3.307(a)(6); §3.307(d); §3.309(e)).
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There are multiple types of amyloidosis, (such as AA, AL, hereditary, and dialysis related), but only AL amyloidosis (light chain/primary), is subject to presumptive service connection under (38 CFR 3.307(a)(6); §3.307(d); §3.309(e)).
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Prior to March 9, 2012, the date this diagnostic code was added to the rating schedule, this condition was evaluated under the analogous diagnostic code of 7799-7715.
Notes
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Assign a permanent 100% evaluation for amyloid light chains (AL) amyloidosis (primary amyloidosis), and grant entitlement to Dependents Educational Assistance (DEA) under 38 U.S.C. Chapter 35, (if not previously established).