A Case of Nephrotic Syndrome due to AHL Amyloidosis in a Patient with Renal Cell Carcinoma

Apostolou T, Maria Smirli and Christina Vourlakou

Department of Nephrology, Evangelismos General Hospital of Athens, Greece
Department of Pathology, Evangelismos General Hospital of Athens, Greece

^*Correspondance to: Apostolou T 

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Abstract:

We report a highly unusual case of a patient with nephrotic syndrome due to AHL primary amyloidosis, with α heavy chain fragments in serum and urine after a recent diagnosis of renal cell carcinoma. The patient was a 52 year-old male with solitary kidney, after nephrectomy of his left kidney, because of Renal Cell Carcinoma (RCC). He was admitted to our hospital, 8 months after nephrectomy, because of severe nephrotic syndrome (total urine protein (TPU) >8.5 gr/24h) with normal renal function. Biopsy of the remaining kidney showed AHL systemic amyloidosis, while immunofixation identified fragments of α-heavy chains in urine and serum, as well as λ light-chains in urine. The diagnosis of primary amyloidosis in which the amyloid deposits are composed of both light and heavy chains, is an extremely unusual occurrence, while only a few cases are presented in the literature. Various studies have showed a rare association of renal cell carcinoma and amyloidosis, but this is related to secondary (AA type) amyloidosis. On the contrary, there are no data where renal carcinomas relate to systemic amyloidosis. Thus this case report is very interesting for two reasons; first for the concurrence between renal carcinoma and systemic amyloidosis and for the heavy chain fragments in serum and urine.

Keywords:

Heavy and light-chain amyloidosis; AHL amyloidosis; Nephrotic syndrome; α-heavy chain; Renal cell carcinoma

Cite the Article:

Apostolou T, Smirli M, Vourlakou C. A Case of Nephrotic Syndrome due to AHL Amyloidosis in a Patient with Renal Cell Carcinoma. Clin Case Rep Int. 2018; 2: 1063.