Plasma cell neoplasia of primary systemic AL amyloidosis type

Abstract

Male white patient, 74 years of age, with a history of hypertension and nephrotic syndrome, probably secondary to membranous glomerulopathy, who was treated by a nephrology specialist. Three months earlier he began with progressive abdominal swelling and epigastric discomfort, unrelated to food intake. He also presented with a moderate amount of postprandial vomiting. Based on all these facts, he was admitted to hospital. In the physical examination, it was noticed the pale straw-colored skin and subcutaneous cellular tissue infiltration in lower limbs up to the knees. The edema was soft, white, cold and with an easy pitting. It was also noticed a painless hepatomegaly which exceeded in 5cm the costal margin of the nodular surface, and an irregular border. The abdominal ultrasound reported a liver with hepatomegaly of 4cm, with four echogenic nodular images with hypoechogenic center. It was established the possibility that the nephrotic syndrome in this patient was of paraneoplastic etiology secondary to prostate cancer with poly-metastatic liver. An interconsultation was requested with the urology specialist. Laparoscopy was performed with no evidence of nodular lesions on the liver surface; however, hepatomegaly was found but without being able to define its etiology. A moderate ascites of milky aspect was also found. After these findings, it was decided to discharge the patient and wait for the results of ten Prostatic Specific Antigen test and prostate biopsy, but it was later reported that the material obtained was insufficient for diagnosis. After being discharged the patient returned to hospital and died at the Emergency Room. At necropsy, morphological findings were determined, that together with the clinical symptoms, allowed the diagnosis of the case as a plasma cell neoplasia of primary systemic AL amyloidosis type.

Keywords

• amiloidosis