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What is Dystrophic calcification means- and explain in detail?
Amyloidosis is the extracellular deposition of insoluble protein fibrils.
AL- Primary systemic amyloidosis
AA- Secondary amyloidosis
AF- Familial
Aβ2M- In ESRD
Diagnosed by abdominal fat pad aspiration showing apple green birefringence by polarized light microscopy with Congo red stain
High ESR, High ALP
Serum Immunofixation electrophoresis (SIFE), UIFE-sensitive to detect light chains (Lamda more common)
Kidneys- most common involved. Nephrotic range proteinuria, azotemia seen
Heart- second common organ; restrictive cardiomyopathy, low voltage complexes in ECG, sparkly appearance on ECHO heart
Peripheral sensory neuropathy,carpal tunnel syndrome
Large tongue, raccoon-eye sign-Factor X deficiency^ bleeding)
Treatment- AL- Melphalan. dexamethasone, lenalidomide & bortezomib;
HDM/SCT- Autologous stem cell transplantation after high-dose melphalan
High BNP. pro-BNP, troponin T & I in cardiac involvement, used for prognosis too.
AA Amyloidosis: secondary to chronic infections - tuberculosis, SABE or inflammatory conditions like RA, IBD, Castleman's disease
Treat the primary disease, TNF & IL -1 antagonist.
The new drug - Eprodisate for renal AA
AF Amyloidosis: AD, mutant TTR (transthyretin/prealbumin)

° Peripheral & autonomic neuropathy
° Cardiac conduction defects

Aβ2M/ Amyloidosis: associated with ESRD. on hemodialysis
Carpal tunnel syndrome, joint effusions, cystic bone lesions
Decreases with stopping dialysis