Primary Plasma Cell Leukemia Associated with Adult Fanconi Syndrome

Primary plasma cell leukemia (PCL) is a rare variant of multiple myeloma that occurs de novo in patients without a history of plasma cell disorder. We describe a case of  PCL that showed a prolonged survival of 2 years and 6 months associated with a generalized dysfunction of the proximal renal tubule. On presentation, the patient had anemia (hemoglobin level, 11 g/dL), thrombocytopenia (platelet count, 102,000/μL), and renal insufficiency (creatinine level, 2.75 mg/dL; creatinine clearance, 19 mL per minute; urea, 71 mg/dL). In addition to the com- mon clinical picture, our case showed proteinuria (30 mg/dL), glycosuria (150 mg/dL) with normal glycemia, low uric acid concentration (2.9 mg/dL), and ion loss from the proximal renal tubule (P, 2.1 mg/dL). These findings were compatible with the clinical picture of adult-acquired Fanconi syndrome, which can be observed in PCL/multiple myeloma but is almost exclusively associated with -chain restriction.