HEMOSTATIC DISORDERS RELATED TO PRIMARY AL AMYLOIDOSIS – A CASEREPORT
Abstract
BACKGROUND Primary AL amyloidosis is characterized by tissue deposits of amyloid, leading to organdysfunction. Acquired hemostatic abnormalities including increased fragility of bloodvessels, platelet dysfunction and coagulation factor deficiencies are common. Improvedoutcome of disease with poor prognosis is shown after a chemotherapy-induced decreasein plasma cell clone and impaired amiloid deposition.Patient and We report about a case of 63 year old women with spontaneus bleedings by who the primethods mary AL amyloidosis was confirmed. She had impaired platelet aggregation, coagulationfactor 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 threecourses 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 thecourse of diseaseDownloads
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