Die mit einem multiplen Myelom assoziierte Immunoglobulin-Leichtketten (AL)-Amyloidose ist unter den systemischen Amyloidosen die häufigste Form, als Entität selbst jedoch relativ selten. Die durch den monoklonalen Plasmazellklon sezernierten instabilen Leichtketten führen als Amyloidablagerungen im Gewebe zu manifesten Organschäden, v. a. die renalen und kardialen Organmanifestationen führen häufig zu einer sehr schlechten Prognose. Besonders kutane Manifestationen wie beidseitige periorbitale Ödeme, Ekchymosen und eine Makroglossie können als pathognomonisch für die systemische AL-Amyloidose betrachtet werden und sollten zu einer raschen Abklärung führen. Wir berichten über einen Patienten mit einer kutanen Manifestation einer AL-Amyloidose mit assoziiertem „smouldering Myelom“.
Abstract
Systemic immunoglobulin light-chain (AL) amyloidosis associated with multiple myeloma is rather common because they both have the same cell of origin. A monoclonal plasma cell clone produces unstable light chains which form amyloid fibrils. These fibrils are then spread through the bloodstream and deposited in the tissue of multiple organs and the skin. Especially renal and cardiac manifestations lead to a very poor outcome for the patients because of the related tissue damage. Focussing on cutaneous manifestations, there are some symptoms like bilateral periorbital haematoma (also known as “raccoon eyes”), ecchymoses and makroglossia, that can be seen as pathognomonic for a systemic AL amyloidosis. Because early diagnosis is essential for the prognosis of the affected patients these signs should lead to a fast diagnostic work-up and treatment. We present a case of a patient with cutaneous manifestations of a systemic AL Amyloidosis associated with a lambda-light-chain smouldering myeloma.
Georg Thieme Verlag KG Rüdigerstraße 14, 70469 Stuttgart, Germany
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