| Abstrakt | | Background Primary AL amyloidosis is characterized by tissue deposits of amyloid, leading to organ dysfunction. Acquired hemostatic abnormalities including increased fragility of blood vessels, platelet dysfunction and coagulation factor deficiencies are common. Improved outcome of disease with poor prognosis is shown after a chemotherapy-induced decrease in plasma cell clone and impaired amiloid deposition. Patient and methods We report about a case of 63 year old women with spontaneus bleedings by who the primary AL amyloidosis was confirmed. She had impaired platelet aggregation, coagulation factor X deficiency, liver, kidney, spleen, peripheral nerves and heart involvement. Because of advanced stage of disease she was not a candidate for autologous stem cell transplantation. She received monthly courses of melphalan and dexamethasone. After three courses only the proteinuria decreased. Because of bleeding diathesis and heart involvement the outcome remains unpredictible. Conclusions For patients with primary AL amyloidosis is important to identify hemostatic disorders. Effect of chemoterapy is often delayed and during that time bleedings may complicate the course of disease.
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