AL-amyloidosis: challenges and perspectives
Резюме. AL-amyloidosis is a dysproteinosis involving various organs and systems, leading to life-threatening organ failure. The disease is a relatively rare and significant problem is late diagnosis, especially cardiac amyloidosis. AL-amyloidosis develops as a result of extracellular deposition of fibrils, which consist of monoclonal light chains of immunoglobulins (the ratio of lambda to kappa is 3:1). Even with a small clone of plasma cells, the development of massive organ damage by these deposits is possible. This condition may be associated with multiple myeloma or other variants of B-lymphoproliferative disease and diagnosed synchronously. Misdiagnosis is often due to the signs and symptoms can mimic the symptoms of more common diseases. Prevention of progressive organ damage is possible due to early diagnosis. Correct staging and determining prognostically significant factors before the start of therapy is extremely important in view of the different therapeutic approaches and determining the strategy for each patient individually. The lack of prospective studies, especially phase III, is one of the biggest problems in the science of treating AL amyloidosis. The treatment regimens for treatment of multiple myeloma, with varying degrees of success, are used as therapy for this disease. The article discusses modern approaches to the diagnosis and treatment of patients with light chain amyloidosis, including standard methods, as well as those that are currently under investigation. Survival of patients with AL-amyloidosis depends on the lesion, the number of organs involved in the pathological process. The most significant is the presence of cardiac amyloidosis and is an unfavorable prognostic sign. However, with the evolution of treatment approaches, a significant proportion of patients achieves therapy success. Assessment of the hematological response is key factor.
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