AL amyloidosis
See also in: External and Internal Eye,Nail and Distal Digit,Oral Mucosal LesionAlerts and Notices
Important News & Links
Synopsis
Primary systemic (AL) amyloidosis is an acquired amyloidosis, almost always associated with a plasma cell dyscrasia. Multiple myeloma is the most common association, but it is also seen with Waldenström macroglobulinemia and other paraproteinemias. The disease is more common in older adults; incidence increases with advancing age. The prognosis is poor.
Amyloid deposits in AL amyloidosis are composed of immunoglobulin light chains. Monoclonal proteins or Bence Jones proteins are usually detected.
Systemic findings can be related to the involvement of the heart, muscles, gastrointestinal tract, kidneys, and nerves. Symptoms may include fatigue, weight loss, paresthesia, syncope, and shortness of breath. Cardiac involvement may present as syncope, angina, or symptoms of heart failure. Low ECG voltages may develop as a consequence of restrictive cardiomyopathy. Renal involvement with nephrotic syndrome is the most common presentation of immunoglobulin amyloidosis. Neurologic symptoms include a sensory peripheral neuropathy, presenting in a stocking and glove distribution. Cognitive impairment and memory loss may be present. An "idiopathic" carpal tunnel syndrome can also occur. Gastrointestinal involvement is variable and may present with hemorrhage, changes in bowel movements, and dyspepsia.
Amyloid infiltration of skeletal muscle can result in pseudohypertrophy, classically involving the tongue and leading to macroglossia.
Mucocutaneous lesions can be seen in 30%-40% of patients with AL amyloidosis and may present before systemic involvement is apparent, providing an early clue to diagnosis.
Abnormal bleeding and/or abnormal bleeding tests are seen in approximately 30%-50% of patients with amyloidosis.
Related topic: AA amyloidosis
Amyloid deposits in AL amyloidosis are composed of immunoglobulin light chains. Monoclonal proteins or Bence Jones proteins are usually detected.
Systemic findings can be related to the involvement of the heart, muscles, gastrointestinal tract, kidneys, and nerves. Symptoms may include fatigue, weight loss, paresthesia, syncope, and shortness of breath. Cardiac involvement may present as syncope, angina, or symptoms of heart failure. Low ECG voltages may develop as a consequence of restrictive cardiomyopathy. Renal involvement with nephrotic syndrome is the most common presentation of immunoglobulin amyloidosis. Neurologic symptoms include a sensory peripheral neuropathy, presenting in a stocking and glove distribution. Cognitive impairment and memory loss may be present. An "idiopathic" carpal tunnel syndrome can also occur. Gastrointestinal involvement is variable and may present with hemorrhage, changes in bowel movements, and dyspepsia.
Amyloid infiltration of skeletal muscle can result in pseudohypertrophy, classically involving the tongue and leading to macroglossia.
Mucocutaneous lesions can be seen in 30%-40% of patients with AL amyloidosis and may present before systemic involvement is apparent, providing an early clue to diagnosis.
Abnormal bleeding and/or abnormal bleeding tests are seen in approximately 30%-50% of patients with amyloidosis.
Related topic: AA amyloidosis
Codes
ICD10CM:
E85.81 – Light chain (AL) amyloidosis
SNOMEDCT:
23132008 – AL amyloidosis
E85.81 – Light chain (AL) amyloidosis
SNOMEDCT:
23132008 – AL amyloidosis
Look For
Subscription Required
Diagnostic Pearls
Subscription Required
Differential Diagnosis & Pitfalls
To perform a comparison, select diagnoses from the classic differential
Subscription Required
Best Tests
Subscription Required
Management Pearls
Subscription Required
Therapy
Subscription Required
References
Subscription Required
Last Reviewed:03/18/2021
Last Updated:08/16/2021
Last Updated:08/16/2021
AL amyloidosis
See also in: External and Internal Eye,Nail and Distal Digit,Oral Mucosal Lesion