Present possibilities of diagnosis and treatment of systemic AL-amyloidosis

Česká verzia

Authors: V. Ščudla; T. Pika
Authors place of work: III. interní klinika Lékařské fakulty UP a FN Olomouc, přednosta prof. MUDr. Vlastimil Ščudla, CSc.
Published in the journal: Vnitř Lék 2009; 55(Suppl 1)(Supplementum 1): 77-87

Summary

The aim of presented communication is summary of actual knowledge in pathogenesis, diagnosis and treatment of primary systemic AL-amyloidosis. Great attention is devoted to contribution of assessment of serum levels of free light chains including κ/λ ratio, measurement of cardiac biomarkers NT-proBNP and troponin, and also ¹³²I-SAP immunoscintigraphy for treatment response monitoring. The necessity of standardized evaluation of treatment results according to International Society for Amyloidosis recommended criteria are discussed. And also current possibilities of conventional chemotherapy, contribution of high‑dose chemotherapy with autologous stem cell transplantation support (HDT-ASCT), inclusively individual “risk adapted” HDT-ASCT considering prognostic factors of the disease were discussed. Present possibility of immunomodulatory therapy with incorporation of thalidomide, bortezomib and lenalidomide, used in case of treatment failure or disease progression, and also supporting care, including organ transplantation are mentioned. The important role of general practitioners and expert internists in diagnosis of early stages of the disease is highlighted – circumstances chiefly determining treatment strategy and prognosis of this severe, formerly difficult to treat and devastating disease.

Key words:
systemic AL-amyloidosis – diagnosis and classification – conventional chemotherapy – autologous stem cell transplantation – prognosis

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