Worldwide Perspectives of Amyloidosis

Vaxman, Iuliana; Gertz, Morie A.

doi:10.1159/000509736

Cited by 3 publications

(3 citation statements)

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“…Mayo Clinic en su algoritmo de tratamiento para pacientes con diagnóstico reciente de amiloidosis, no candidatos a trasplante de medula ósea, considera el uso del esquema CyBorD (Ciclofosfamida, Bortezomib, Dexametasona) [12]. Este esquema terapéutico fue instaurado como tratamiento específico inicial en este caso.…”

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Caso Clínico: Síndrome nefrótico como presentación de amiloidosis AL, una asociación poco frecuente en nuestro medio

Ordoñez¹,

Cabrera²,

Montero³

2022

Rev Med HJCA

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BACKGROUND: Amyloidosis is a heterogeneous group of diseases with multi-organ involvement and different clinical manifestations; one of them is nephrotic syndrome. Amyloidosis results from the extracellular deposit of fibrillar proteins that form the amyloid substance; amyloidogenic proteins resistant to proteolysis infiltrate tissues extracellularly and are capable of severely altering their structure and function. Amyloid deposits in the renal parenchyma can be observed in the mesangium, capillary wall, interstitium, and arterial vessels. Its diagnosis requires a biopsy of the comprised organs. CASE REPORT: 53-year-old male patient, presented with the incidental finding of microalbuminuria. The only clinical manifestations were mild lower limbs edema and foamy urine. Laboratory tests showed: hypoalbuminemia, hypercholesterolemia, hypertriglyceridemia and nephrotic- range proteinuria. With pure nephrotic syndrome diagnosis a renal biopsy was performed, leading to the histopathological diagnosis of amyloidosis. EVOLUTION: Nephroprotective measures were instituted and the extent of systemic involvement was studied, we diagnosed congestive heart failure and amyloid deposition in the digestive system. The patient initially received treatment based on Cyclophosphamide, Bortezomib and dexamethasone, with no renal or haematological response after four cycles of treatment, so treatment was changed to Bortezomib and Bendamustine. CONCLUSION: It is vitally important to have a high index of suspicion and to make an early diagnosis of the disease through a biopsy with Congo red staining. The treatment depends on the type of amyloidosis, its opportune start allows to increase survival rate, as well as, to avoid the deposit of amyloid in other organs.

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Section: Discussionunclassified

“…Estudios han demostrado un tiempo medio desde el diagnóstico hasta inicio de diálisis, de 14 meses y desde ésta hasta la muerte 8 meses. Siendo la afectación cardíaca (insuficiencia cardíaca y arritmias) la principal causa de muerte, seguido de enfermedad renal [12].…”

Section: Discussionunclassified

Caso Clínico: Síndrome nefrótico como presentación de amiloidosis AL, una asociación poco frecuente en nuestro medio

Ordoñez¹,

Cabrera²,

Montero³

2022

Rev Med HJCA

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“…Despite the pandemic, we followed our tradition of publishing special issues dedicated to one topic. In 2020, we published a special issue entitled “Light Chain Amyloidosis” edited by Dr. Iuliana Vaxman and Dr. Morie A. Gertz [8] which covered worldwide perspectives of amyloidosis. My sincere thanks to both editors for providing a comprehensive overview on amyloidosis.…”

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confidence: 99%