Table 1.

Teaching points

The association of pancytopenia and hepatomegaly or splenomegaly in the context of primary hyperoxaluria is suggestive of bone marrow failure due to crystals deposition and extramedullary hematopoiesis.

Pancytopenia in the context of medullary oxalosis is usually not responsive to stimulating agents and might require iterative transfusions and be responsible of specific complications as in this case, where leucopenia might have contributed to the occurrence of sepsis.

RNA interference therapy should thus be considered as a first-line treatment in every case of primary oxalosis, considering the significant morbidity associated with liver transplantation, and could have prevented the aggravation of bone marrow failure under immunosuppressive drugs as in the case of our patient.