Advances in Clinical and Experimental Medicine

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Advances in Clinical and Experimental Medicine

2011, vol. 20, nr 6, November-December, p. 771–778

Publication type: review article

Language: English

AL Amyloidosis (Amyloidosis Antibody Light). Part 2 – Epidemiology, Clinical Symptoms, Diagnosis and Treatment of Amyloidosis AL

Amyloidoza AL (amyloidosis antibody light). Część II – epidemiologia, objawy kliniczne, rozpoznanie i leczenie amyloidozy AL

Lidia Usnarska-Zubkiewicz1,, Jadwiga Hołojda2,, Kazimierz Kuliczkowski1,

1 Department of Hematology, Blood Neoplasms and Bone Marrow Transplantation, Wroclaw Medical University, Poland

2 Hematology Department of District Specialist Hospital of Legnica, Poland

Abstract

Studies have shown that 10–20% of patients with multiple myeloma develop AL amyloidosis. AL amyloidosis can affect the kidney and the heart, as well as cause peripheral polyneuropathy and CNS impairment. The main diagnostic test to confirm amyloidosis is the staining of the adipose tissue from abdominal fat with Congo red, which demonstrates green birefringence when polarized light is used, or reveals nonbranching fibril structures with a diameter of 10 nm under an electron microscope. Oral melphalan and prednisone have been the leading drugs in the therapy of AL amyloidosis for 30 years. The patients qualified for megachemotherapy and stem cells transplantation had to be free from severe, burdening diseases and had to have less than three involved organs.

Streszczenie

Badania wykazały, że u 10–20% pacjentów chorych na szpiczaka mnogiego rozwija się amyloidoza AL. W przebiegu tej postaci skrobiawicy najczęściej dochodzi do zajęcia nerek i serca oraz rozwoju polineuropatii obwodowej i uszkodzenia o.u.n. Podstawowym testem diagnostycznym potwierdzającym amyloidozę jest barwienie czerwienią Kongo tkanki tłuszczowej z fałdu brzusznego i wykazanie charakterystycznego zielonego, dwułomnego świecenia w świetle spolaryzowanym lub nierozgałęzionych włóknistych struktur o średnicy 10 nm w mikroskopie elektronowym. Od 30 lat wiodącymi lekami w terapii amyloidozy AL są melfalan i prednison. Podobnie jak w leczeniu szpiczaka mnogiego, w terapii amyloidozy zaproponowano stosowanie talidomidu, lenalidomidu i bortezomibu, w monoterapii lub w połączeniu z deksametazonem. Do megachemioterapii i przeszczepienia komórek macierzystych byli kwalifikowani chorzy bez poważnych, obciążających chorób, którzy mieli zajęte przez amyloidozę mniej niż trzy narządy.

Key words

amyloidosis AL, epidemiology, clinical symptoms, diagnosis, treatment

Słowa kluczowe

amyloidoza AL, epidemiologia, objawy kliniczne, rozpoznanie, leczenie

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