Résumé
A 31-year-old male born of a second degree consanguineous union was admitted with fatigue for 3 months. His past medical history was significant for recurrent renal stones from the age of 12 years. He was evaluated and diagnosed to have primary hyperoxaluria. He developed renal failure secondary to nephrolithiasis and is on hemodialysis for past 6 years. His younger brother also has renal failure secondary to nephrolithiasis. Examination showed hepatosplenomegaly. His hemoglobin was 8.9 g/dl (normocytic, normochromic), total white blood cell count 4900/μl, platelet count 1.1 × 109/L, erythrocyte sedimentation rate 88 mm in 1 h. Peripheral smear showed dimorphic anemia, anisopoikilocytosis, polychromasia and mild thrombocytopenia. He had normal blood sugars and altered renal function tests. Liver function tests showed only hypoalbuminemia. A bone marrow biopsy revealed extensive eosinophilic needle shaped crystalline structures arranged in radial pattern replacing normal hematopoietic cells [Figure 1a and b], which showed birefringence under polarized light [Figure 1c and d] consistent with calcium oxalate monohydrate crystals. Primary hyperoxaluria is an inherited disorder featured by the hyperoxaluria, calcium oxalate urinary lithiasis in childhood, nephrocalcinosis, and renal failure. Bone marrow oxalate deposition can be associated with variable degrees of cytopenias, leukoerythroblastic