Sandbox:Sahar
Amyloisosis subtype | Protein | Organ involvement (%) | ||
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Kidney | Liver | Heart | ||
Primary | Monoclonal immunoglobulin light chains | 50-100 | 10-25 | 50-100 |
Secondary | Serum amyloid A | 50-100 | <10 | _ |
DRA (dialysis related amyloidosis) | Beta‐2 microglobulin | _ | _ | _ |
Senile systemic (cardiac) amyloidosis | Transthyretin (wild type) | _ | _ | 50-100 |
Meretoja syndrome | Gelsolin | _ | _ | _ |
Infection/Inflammation | |||||||||||||||||||||||
Increased production of IL-1/IL-6/TNF-α | |||||||||||||||||||||||
Upregulation of hepatic serum amyloid A production | |||||||||||||||||||||||
SAA production uptake by macrophages | |||||||||||||||||||||||
C-terminal cleavage of SAA | |||||||||||||||||||||||
β-sheet configuration of SAA | |||||||||||||||||||||||
Fibril deposition in extracellular space | |||||||||||||||||||||||
Binding of glycosaminoglycan, serum amyloid P, and lipid components | |||||||||||||||||||||||
Resistant to proteolysis | |||||||||||||||||||||||
The above algorithm is adopted from International Journal of Nephrology and Renovascular Disease |
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Organ System Involvement | Differential Diagnosis | Causes | Clinical Features | Laboratory Findings | Gold Standard Test | Therapy |
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Nephrotic Syndrome and Renal Failure | Secondary (AA) Amyloidosis |
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Primary (AL) Amyloidosis |
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Diabetic Nephropathy |
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Minimal Change Disease |
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Focal Segmental Glomerulosclerosis | ||||||
Fabry's Disease |
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Light Chain Deposition Disease |
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Membranous Glomerulonephritis |
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Fibrillary-Immunotactoid Glomerulopathy |
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Organ System Involvement | Differential Diagnosis | Causes | Clinical Features | Laboratory Findings | Gold Standard Test | Therapy |
Polyneuropathy | POEMS syndrome (Demyelinating) |
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Metabolic Syndrome (Axonal pathology) |
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Vitamin Deficiencies (Axonal Pathology) |
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Guillain-Barre Syndrome (Demyelinating) |
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Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) (Mixed axonal and demyelinatiing) |
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Multifocal Motor Neuropathy |
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Organ System Involvement | Differential Diagnosis | Causes | Features | Laboratory Findings | Gold Standard Test | Therapy |
Organomegaly (Hepatosplenomegaly and Lymphadenopathy) | Malaria |
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Kala-azar |
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Infective Hepatitis |
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Chronic Myelogenous Leukemia (CML) |
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Lymphoma |
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Primary (AL) Amyloidosis |
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Gaucher's Disease |
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Example #1
The patient presented with S.O.B. one year after hysterectomy for a leiomyomatous uterus.