AL amyloidosis: Difference between revisions
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{{Infobox medical condition | |||
| name = AL amyloidosis | |||
| synonyms = Primary amyloidosis | |||
| field = [[Hematology]] | |||
| symptoms = [[Fatigue]], [[weight loss]], [[edema]], [[neuropathy]], [[cardiomyopathy]], [[nephrotic syndrome]] | |||
| complications = [[Heart failure]], [[kidney failure]], [[peripheral neuropathy]] | |||
| onset = Typically in [[adulthood]] | |||
| duration = [[Chronic (medicine)|Chronic]] | |||
| causes = [[Monoclonal gammopathy]] | |||
| risks = [[Multiple myeloma]], [[Waldenström's macroglobulinemia]] | |||
| diagnosis = [[Biopsy]], [[immunofixation electrophoresis]], [[serum free light chain analysis]] | |||
| differential = [[AA amyloidosis]], [[hereditary amyloidosis]], [[senile systemic amyloidosis]] | |||
| treatment = [[Chemotherapy]], [[autologous stem cell transplantation]], [[supportive care]] | |||
| prognosis = Variable, depends on organ involvement | |||
| frequency = Rare | |||
}} | |||
'''AL Amyloidosis''' is a rare disease characterized by the deposition of abnormal proteins, known as [[amyloid]] proteins, in various tissues and organs of the body. The disease is also known as '''primary amyloidosis''' or '''light chain amyloidosis'''. | '''AL Amyloidosis''' is a rare disease characterized by the deposition of abnormal proteins, known as [[amyloid]] proteins, in various tissues and organs of the body. The disease is also known as '''primary amyloidosis''' or '''light chain amyloidosis'''. | ||
==Etiology== | ==Etiology== | ||
AL Amyloidosis is caused by the overproduction of [[immunoglobulin light chain]] (a part of the immune system's antibodies) by the [[plasma cells]] in the [[bone marrow]]. These light chains misfold and form amyloid fibrils, which deposit in tissues and organs, causing damage and dysfunction. | AL Amyloidosis is caused by the overproduction of [[immunoglobulin light chain]] (a part of the immune system's antibodies) by the [[plasma cells]] in the [[bone marrow]]. These light chains misfold and form amyloid fibrils, which deposit in tissues and organs, causing damage and dysfunction. | ||
==Symptoms== | ==Symptoms== | ||
The symptoms of AL Amyloidosis vary depending on the organs affected. Common symptoms include fatigue, weight loss, shortness of breath, and swelling in the legs and ankles. If the heart is affected, it can lead to [[congestive heart failure]]. If the kidneys are affected, it can lead to [[nephrotic syndrome]] and kidney failure. | The symptoms of AL Amyloidosis vary depending on the organs affected. Common symptoms include fatigue, weight loss, shortness of breath, and swelling in the legs and ankles. If the heart is affected, it can lead to [[congestive heart failure]]. If the kidneys are affected, it can lead to [[nephrotic syndrome]] and kidney failure. | ||
==Diagnosis== | ==Diagnosis== | ||
Diagnosis of AL Amyloidosis involves a combination of blood tests, urine tests, imaging studies, and tissue biopsy. The [[Congo red stain]] is often used in tissue biopsy to confirm the presence of amyloid proteins. | Diagnosis of AL Amyloidosis involves a combination of blood tests, urine tests, imaging studies, and tissue biopsy. The [[Congo red stain]] is often used in tissue biopsy to confirm the presence of amyloid proteins. | ||
==Treatment== | ==Treatment== | ||
Treatment for AL Amyloidosis aims to reduce the production of light chains and remove the amyloid deposits. This can involve chemotherapy, stem cell transplantation, and supportive care to manage symptoms. | Treatment for AL Amyloidosis aims to reduce the production of light chains and remove the amyloid deposits. This can involve chemotherapy, stem cell transplantation, and supportive care to manage symptoms. | ||
==Prognosis== | ==Prognosis== | ||
The prognosis of AL Amyloidosis depends on the extent of organ involvement and the patient's response to treatment. Early diagnosis and treatment can improve the prognosis. | The prognosis of AL Amyloidosis depends on the extent of organ involvement and the patient's response to treatment. Early diagnosis and treatment can improve the prognosis. | ||
==See also== | ==See also== | ||
* [[Amyloidosis]] | * [[Amyloidosis]] | ||
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* [[Nephrotic syndrome]] | * [[Nephrotic syndrome]] | ||
* [[Congo red stain]] | * [[Congo red stain]] | ||
[[Category:Rare diseases]] | [[Category:Rare diseases]] | ||
[[Category:Protein folding disorders]] | [[Category:Protein folding disorders]] | ||
[[Category:Immunology]] | [[Category:Immunology]] | ||
[[Category:Hematology]] | [[Category:Hematology]] | ||
{{medicine-stub}} | {{medicine-stub}} | ||
{{No image}} | {{No image}} | ||
Latest revision as of 22:19, 3 April 2025
| AL amyloidosis | |
|---|---|
| Synonyms | Primary amyloidosis |
| Pronounce | N/A |
| Specialty | N/A |
| Symptoms | Fatigue, weight loss, edema, neuropathy, cardiomyopathy, nephrotic syndrome |
| Complications | Heart failure, kidney failure, peripheral neuropathy |
| Onset | Typically in adulthood |
| Duration | Chronic |
| Types | N/A |
| Causes | Monoclonal gammopathy |
| Risks | Multiple myeloma, Waldenström's macroglobulinemia |
| Diagnosis | Biopsy, immunofixation electrophoresis, serum free light chain analysis |
| Differential diagnosis | AA amyloidosis, hereditary amyloidosis, senile systemic amyloidosis |
| Prevention | N/A |
| Treatment | Chemotherapy, autologous stem cell transplantation, supportive care |
| Medication | N/A |
| Prognosis | Variable, depends on organ involvement |
| Frequency | Rare |
| Deaths | N/A |
AL Amyloidosis is a rare disease characterized by the deposition of abnormal proteins, known as amyloid proteins, in various tissues and organs of the body. The disease is also known as primary amyloidosis or light chain amyloidosis.
Etiology[edit]
AL Amyloidosis is caused by the overproduction of immunoglobulin light chain (a part of the immune system's antibodies) by the plasma cells in the bone marrow. These light chains misfold and form amyloid fibrils, which deposit in tissues and organs, causing damage and dysfunction.
Symptoms[edit]
The symptoms of AL Amyloidosis vary depending on the organs affected. Common symptoms include fatigue, weight loss, shortness of breath, and swelling in the legs and ankles. If the heart is affected, it can lead to congestive heart failure. If the kidneys are affected, it can lead to nephrotic syndrome and kidney failure.
Diagnosis[edit]
Diagnosis of AL Amyloidosis involves a combination of blood tests, urine tests, imaging studies, and tissue biopsy. The Congo red stain is often used in tissue biopsy to confirm the presence of amyloid proteins.
Treatment[edit]
Treatment for AL Amyloidosis aims to reduce the production of light chains and remove the amyloid deposits. This can involve chemotherapy, stem cell transplantation, and supportive care to manage symptoms.
Prognosis[edit]
The prognosis of AL Amyloidosis depends on the extent of organ involvement and the patient's response to treatment. Early diagnosis and treatment can improve the prognosis.
