Primary systemic amyloidosis

Background. Systemic amyloidosis is a rare disorder which usually occurs in aged persons and has a poor prognosis. Systemic amyloidosis can be primary, occasionally associated with multiple myeloma, or secondary, associated with another disease. Case report. We presented a 72-year-old male patient w...

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Main Authors: Tanasilović Srđan, Živanović Dubravka, Nikolić Miloš, Tomović Maja, Elezović Ivo, Medenica Ljiljana
Format: Article
Language:English
Published: Ministry of Defence of the Republic of Serbia, University of Defence, Belgrade 2007-01-01
Series:Vojnosanitetski Pregled
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Online Access:http://www.doiserbia.nb.rs/img/doi/0042-8450/2007/0042-84500712859T.pdf
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author Tanasilović Srđan
Živanović Dubravka
Nikolić Miloš
Tomović Maja
Elezović Ivo
Medenica Ljiljana
author_facet Tanasilović Srđan
Živanović Dubravka
Nikolić Miloš
Tomović Maja
Elezović Ivo
Medenica Ljiljana
author_sort Tanasilović Srđan
collection DOAJ
description Background. Systemic amyloidosis is a rare disorder which usually occurs in aged persons and has a poor prognosis. Systemic amyloidosis can be primary, occasionally associated with multiple myeloma, or secondary, associated with another disease. Case report. We presented a 72-year-old male patient with periocular purpura ("racoon sign") and waxy papules, petechiae and ecchymoses on the neck and thoracic area. Purpuric macules were present also on the lips and tongue which was edematous (macroglossia). The skin lesions occurred two years earlier, the patient lost more than 15 kilograms of the body mass for less than a year. Immunoelectrophoresis of urine and serum demonstrated the presence of immunoglobulin light chains of the circulating monoclonal protein. Histopathological examination of skin lesions showed Congo red positive deposits in the derm. Cardiac evaluation revealed the signs of heart failure, and renal evaluation revealed nephrotic syndrome, with excessive protein lost. He was treated with oral melphalan and prednisolone, and died 7 days after starting the therapy due to heart failure. Conclusion. This patient considered as a rare case with systemic amyloidosis highlights the importance of histopathological and physical examination in any cases with periocular purpura, petechiae, ecchymoses and macroglossia.
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spelling doaj-art-2b3941d726ee41618aaf4305cabc1dc92025-08-20T02:04:10ZengMinistry of Defence of the Republic of Serbia, University of Defence, BelgradeVojnosanitetski Pregled0042-84502007-01-01641285986210.2298/VSP0712859TPrimary systemic amyloidosisTanasilović SrđanŽivanović DubravkaNikolić MilošTomović MajaElezović IvoMedenica LjiljanaBackground. Systemic amyloidosis is a rare disorder which usually occurs in aged persons and has a poor prognosis. Systemic amyloidosis can be primary, occasionally associated with multiple myeloma, or secondary, associated with another disease. Case report. We presented a 72-year-old male patient with periocular purpura ("racoon sign") and waxy papules, petechiae and ecchymoses on the neck and thoracic area. Purpuric macules were present also on the lips and tongue which was edematous (macroglossia). The skin lesions occurred two years earlier, the patient lost more than 15 kilograms of the body mass for less than a year. Immunoelectrophoresis of urine and serum demonstrated the presence of immunoglobulin light chains of the circulating monoclonal protein. Histopathological examination of skin lesions showed Congo red positive deposits in the derm. Cardiac evaluation revealed the signs of heart failure, and renal evaluation revealed nephrotic syndrome, with excessive protein lost. He was treated with oral melphalan and prednisolone, and died 7 days after starting the therapy due to heart failure. Conclusion. This patient considered as a rare case with systemic amyloidosis highlights the importance of histopathological and physical examination in any cases with periocular purpura, petechiae, ecchymoses and macroglossia.http://www.doiserbia.nb.rs/img/doi/0042-8450/2007/0042-84500712859T.pdfamyloidosissigns and symptomspurpuramacroglossiadiagnosisdrug therapytreatmentoutcome
spellingShingle Tanasilović Srđan
Živanović Dubravka
Nikolić Miloš
Tomović Maja
Elezović Ivo
Medenica Ljiljana
Primary systemic amyloidosis
Vojnosanitetski Pregled
amyloidosis
signs and symptoms
purpura
macroglossia
diagnosis
drug therapy
treatment
outcome
title Primary systemic amyloidosis
title_full Primary systemic amyloidosis
title_fullStr Primary systemic amyloidosis
title_full_unstemmed Primary systemic amyloidosis
title_short Primary systemic amyloidosis
title_sort primary systemic amyloidosis
topic amyloidosis
signs and symptoms
purpura
macroglossia
diagnosis
drug therapy
treatment
outcome
url http://www.doiserbia.nb.rs/img/doi/0042-8450/2007/0042-84500712859T.pdf
work_keys_str_mv AT tanasilovicsrđan primarysystemicamyloidosis
AT zivanovicdubravka primarysystemicamyloidosis
AT nikolicmilos primarysystemicamyloidosis
AT tomovicmaja primarysystemicamyloidosis
AT elezovicivo primarysystemicamyloidosis
AT medenicaljiljana primarysystemicamyloidosis