INTRODUCTION: The epidemiology of amyloidosis is not well known and its diagnosis is difficult, due to unspecific early clinical manifestations. Amyloidosis is considered when organ failure occurs. Renal, cardiac and hepatic involvement usually occurred despite therapy. Presenting this case report, we aimed to underline the need of new treatments for amyloidosis. MATERIALS AND METHODS: We describe a 41 years old woman admitted to the hospital with proteinuria (1280 mg/24h) and rapid deterioration of renal function (serum creatinine from 0.8 to 1.6 mg/dL). Autoantibodies, immunoglobulin and C3/C4 were negative. A renal biopsy showed the presence of AL-Amyloidosis with k-light chains deposition at immunofluorence. Subsequently, the patient showed nephrotic syndrome onset (proteinuria 4000 mg/24h with albuminuria 3400 mg/24h) and increased rates of cholestasis with hepatomegaly and hepatic failure. RESULTS: Treatment with bortezomib and dexamethasone gave a complete hematological response but renal function was not improved. DISCUSSION: This case is very interesting because renal involvement was the initial presentation of amyloidosis and rapid progressive renal and hepatic failure was subsequentely observed; its management was challenging from the clinical approach to the final diagnosis and treatment. CONCLUSIONS: In terms of organ response, it is necessary to develop new strategies to counteract the progressive organ failure due to amyloid deposition.

Rapidly Progressive Renal and Hepatic Failure in AL-Amyloidosis: Bortezomib and Steroid Support in a Young Woman Two Months After Delivery / Fiacco, Federica; Umbro, Ilaria; Zavatto, Assunta; Di Natale, V.; D’Amici, S.; Tinti, Francesca; Mitterhofer, Anna Paola. - In: CELLR4. - ISSN 2329-7042. - ELETTRONICO. - (2014).

Rapidly Progressive Renal and Hepatic Failure in AL-Amyloidosis: Bortezomib and Steroid Support in a Young Woman Two Months After Delivery

FIACCO, FEDERICA;UMBRO, ILARIA;ZAVATTO, ASSUNTA;D’Amici S.;TINTI, FRANCESCA;MITTERHOFER, Anna Paola
2014

Abstract

INTRODUCTION: The epidemiology of amyloidosis is not well known and its diagnosis is difficult, due to unspecific early clinical manifestations. Amyloidosis is considered when organ failure occurs. Renal, cardiac and hepatic involvement usually occurred despite therapy. Presenting this case report, we aimed to underline the need of new treatments for amyloidosis. MATERIALS AND METHODS: We describe a 41 years old woman admitted to the hospital with proteinuria (1280 mg/24h) and rapid deterioration of renal function (serum creatinine from 0.8 to 1.6 mg/dL). Autoantibodies, immunoglobulin and C3/C4 were negative. A renal biopsy showed the presence of AL-Amyloidosis with k-light chains deposition at immunofluorence. Subsequently, the patient showed nephrotic syndrome onset (proteinuria 4000 mg/24h with albuminuria 3400 mg/24h) and increased rates of cholestasis with hepatomegaly and hepatic failure. RESULTS: Treatment with bortezomib and dexamethasone gave a complete hematological response but renal function was not improved. DISCUSSION: This case is very interesting because renal involvement was the initial presentation of amyloidosis and rapid progressive renal and hepatic failure was subsequentely observed; its management was challenging from the clinical approach to the final diagnosis and treatment. CONCLUSIONS: In terms of organ response, it is necessary to develop new strategies to counteract the progressive organ failure due to amyloid deposition.
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/11573/614184
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