SUN-390 MIXED AMYLOSE AA AND AL

Abstract

Systemic amyloidosis is a rare disease that can affect multiple organs. Renal involvement is the most common and the most clinically expressive. Its diagnosis is histological based on the presence of amyloid deposits which are most often either type AA essentially complicating infections and chronic inflammatory rheumatism, either of primitive AL type or during dysglobulinemia. This presentaion is about the first case in ou the first case of mixed amyloidosi AL and AA. Descriptive presentation of the first mixed amylosis AA and AL that occured in the nephrology departement of Sahloul hospital in 2019. The case of a 66-year-old man with a history of treated and cured tuberculosis, gout and benign prostatic hypertrophy who was admitted to primary renal insufficiency with a creatinine count of 1078 was presented. Examination the patient had a small tension and clinical splenomegaly. In biology were found bi cytopenia with normochromic normocytic anemia, leukopenia and nephrotic syndrome. Plasma protein electrophoresis was performed and showed hypogammaglobulinemia at 4.9 with a monoclonal peak estimated at 2.1 g / l of gamma globulins, complement by imminotyping was in favor of an IgG Lamda type monoclonal peak. Sternal puncture was performed and the diagnosis of myeloma was eliminated with a plasma cell count estimated at 6% and negative CRAB criteria. To confirm the diagnosis of amyloidosis, in front of the appearance of small kidneys, the only recourse was to perform a biopsy of accessory salivary glands which had confirmed the diagnosis of amyloidosis with histochemistry SAA (+) Kappa (-) Lamda (+). Series of complementary tests whose tumor markers were measured and showed only an increased ACE level. However, upper digestive fibroscopy and colonoscopy were normal. The patient was treated with dialysis at a rate of two sessions per week. The course was marked by a general condition preserved with onset during hospitalization of a periorbital bruise. In view of the imminilogical clinical arguments and the etiological investigation the diagnosis retained was mixed amyloidosis as a new entity and the end stage renal failure patient was adressed to hematology to manage his gammopathy. AA amyloidosis is deposited during tuberculosis, a major cause in developing countries. In our case and after eliminating a secondary cause, that was the probable cause of this AA amyloidosis. However, the clinical and biological examination did not show any activity of this tuberculosis. The patient had also a monoclonal peak of IgG lamda type with a 6% plasma cells level at the sternal puncture. This suggests the presence of a monoclonal gammopathy at the origin of AL amyloidosis especially with the appearance on clinical examination of peri-orbital eccymosis. The diagnosis of the probability of AL amyloidosis is sometimes focused on a set of arguments: clinical context compatible with AL amyloidosis, presence of serum / urinary monoclonal gammopathy or a clonal medullary plasma cell population with light chain assay. We suggest, after all these arguments the presence in this patient the first case of a mixed amyloidosis AA and AL. The techniques for identifying the nature of amyloid deposits by proteomics, currently under development, should allow in the future to identify precisely the type of amyloidosis in the majority of cases.