Primary (AL) | Monoclonal light chains (clonal or frankly malignant plasma cells) |
death within 6–12 months of symptom onset multiple myeloma non-Hodgkin’s lymphoma Waldenstrom’s macroglobulinemia diagnostic sensitivity of fat aspiration 84% |
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Senile systemic (ATTRwt) | Wild-type transthyretin |
without underling diseases diagnostic sensitivity of fat aspiration 15% (11–20%) |
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Hereditary (ATTRv) | Mutant transthyretin (more than 120 mutations) |
death within 5–15 years of symptom onset without underling diseases inherited in an autosomal dominant fashion diagnostic sensitivity of fat aspiration 45% (36–54%), but dependent on mutation ( |
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Secondary (AA) | Kidneys, GI tract, |
Serum amyloid A |
infectious, inflammatory, or neoplastic insults rheumatoid arthritis ankylosing spondylitis severe gout tuberculosis, bronchiectasis osteomyelitis inflammatory bowel disease Hodgkin’s disease renal-cell carcinoma diagnostic sensitivity of fat aspiration - not analyzed |
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Dialysis-related (Aβ2M) | Osteoarticular tissue, GI tract, circulatory system, |
β2-microglobulin |
renal failure with dialysis effective treatment is renal transplantation |
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Isolated atrial (AANF) | Atrial natriuretic factor |
abnormal heart rhythms |
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Hereditary (ALys) | Kidneys, liver | Lysozyme |
without underling diseases |
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Hereditary (AFib) | Kidneys, liver | Mutant fibrinogen A α |
without underling diseases |
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Hereditary (AApoA1, 2) | Principal component of high-density lipoprotein- |
without underling diseases |
Biventricular HF especially with HFpEF | Macroglossia and periorbital purpura (AL type) |
Newly diagnosed HCMP in elderly patients | Carpal tunnel syndrome, particularly if bilateral |
HFpEF associated with nephrotic syndromes | Spinal stenosis (mainly ATTR wt) |
Low normal blood pressure with previous history of hypertension | Autonomic signs and symptoms (orthostatic hypotension, alternating constipation/diarrhea, sweating abnormalities) associated with peripheral neuropathy (both types) |
Intolerance to beta blocker, ACEi, or ARB, ARNI | Spontaneous biceps tendon rupture |
Unexplained conduction block needing pacemaker AV block + increased LV wall thickness | Nephrotic syndrome/non-diabetic proteinuria (mainly AL type) |
Newly diagnosed low flow, low gradient aortic stenosis in elderly patients | Hepatic alteration/hepatomegaly disproportionated to HF status (mainly AL type) |
Diuretic therapy Conventional heart failure treatment (use with caution) Anticoagulation Devices and ablation therapy Cardiac assist devices Heart transplantation |
Conventional chemotherapy: alkylating agents, proteasome inhibitors, immunomodulators, immunotherapy High-dose chemotherapy and autologous stem cell transplantation |
Drugs that stabilize the transthyretin tetramer: Tafamidis, Diflunisal Drugs that inhibit TTR gene expression: Inotersen, Patisiran Drugs that inhibit oligomer aggregation and tetramer dissociation: Epigallocatechin-3-gallate Drugs that affect degradation and reabsorption of amyloid fibers: Doxycycline, Tauroursodeoxycholic acid, Miridesap, Dezamizumab Orthotopic liver transplantation Liver-heart transplantation |