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[Systemic amyloidosis as a cause of so-called atherosclerotic hemorrhagic complications]
A Sakalová1, D Skultétyová, J Skultéty
1Klinika hematológie a transfuziológie FN, Bratislava.
Background:
A survey of the pathogenesis, diagnostics and treatment of amyloidosis is presented, illustrated by long-term clinical experience with the treatment of 28 patients with primary amyloidosis, 4 patients with familial and 7 patients with secondary amyloidosis.
Methods And Results:
To all patients combined treatment of glucocorticosteroids and cytostatics was administered. For primary amyloidosis methylprednisolone, vincristine (oncovine), cyclosphosphamide, alkeran, chlorethylnitrosourea (MOCCA), and in secondary amyloidosis cyclophosphamide, vincristine, prednisone (CVP). In all patients, this treatment was combined with dimethylsulphoxide treatment (DMSO) administered in intravenous infusion (5 g/100 ml saline) for 3-5 days before the start of cytostatic therapy. In primary amyloidosis the median of survival was 48 months, in secondary amyloidosis the median survival was 72 months.
Conclusions:
The authors emphasise the need of an early diagnosis and start of comprehensive therapy completed in recent years successfully with immunomodulatory therapy using hydrolytic enzymes (WOBE MUGOS).