Background: Extramedullary plasmacytoma is an immunoproliferative monoclonal disease of the B-cell line that originates from malignant transformed plasma cells. It’s a rare variant of plasma cell tumor involving organs outside the bone marrow, found in 13% of cases. Urinary bladder involvement remains a location rarely reported in the literature. Herein, we report a case of bladder extramedullary plasmacytoma revealing multiple myeloma. Case report: A 49-year-old man presented to the urology department with an acute obstructive renal failure revealed by renal colic and dysuria gradually evolving over 2 months. The renal ultrasound and the pelvic abdominal CT scan revealed a bilateral ureter hydronephrosis with a locally advanced bladder tumor. The patient underwent surgical resection of the tumor and the histopathology study of surgical specimen showed an infiltration of the urothelial mucosa by abnormal plasma cells. The bone marrow aspirate established the presence of 59% of abnormal plasma cells and rouleaux formation of red blood cells, serum protein electrophoresis showed a monoclonal peak in the area of gamma globulin, and the conventional radiography of the axial skeleton objectifies lytic spine lesions. The diagnosis of a multiple myeloma associated to an extramedullary bladder location was retained. The outcome was unfavorable and the patient died of severe pulmonary embolism before initiating any therapy. Conclusion: Secondary extramedullary plasmacytoma is a marker of poor prognosis in both newly diagnosed and relapsed multiple myeloma patients. Bladder location remains a rare entity, the clinical presentation is nonspecific and management constitutes a therapeutic challenge even in the era of new agents.

Introduction: Glycogen storage disease type I is a rare hereditary metabolic disorder, caused by a deficit of the glucose-6-phosphatase, an enzyme that catalyzes hydrolysis of glucose-6-phosphate to phosphate and glucose, inducing essentially hypoglycemia, hyperlactacidemia, hyperuricemia and hyperlipoproteinemia often at the expense of triglycerides. Hypertriglyceridemia is often moderate (<10 g / l) at the time of diagnosis, but in the long term, it can exceed 20g/l causing serious complications such as acute pancreatitis or hepatic adenoma. We report a case of glycogen storage disease type I revealed by major hypertriglyceridemia.Case report: A 10-month-old infant girl, with a family history of storage disease type I (brother), admitted to the pediatric department for recurrent seizures due to hypoglycemia evolving for 2 months, and hepatomegaly. The blood tests made on admission revealed a hepatic cytolysis with a liver enzyme level 5 times upper the limit of normal, a major hypertriglyceridemia at 10 g / l (<1.5 g / l), and a correct glycemia and uricaemia. The infant was put on an adequate diet therapy. The evolution was marked by good clinical improvement.Conclusion: Major hypertriglyceridemia is a metabolic abnormality often occurring during the course of glycogen storage disease type I, but sometimes it is discovered at the time of diagnosis requiring prompt management and strict monitoring to avoid complications.