Unexplained Fatal Hyperammonemia in a Patient With New Diagnosis of Acute Monoblastic Leukemia

Idiopathic hyperammonemia is a serious condition that can arise after induction of chemotherapy and is characterized by plasma ammonia levels greater than two times the normal upper limit but within the context of normal liver function. While this dangerous complication usually appears several weeks after the start of chemotherapy, we report a fatal case of idiopathic hyperammonemia that was detected only nine days after induction chemotherapy in a 22-year-old man with no liver pathology or other risks for hyperammonemia. The patient's initial emergent presentation was altered mental status. Laboratory workup showed acute monoblastic leukemia and radiological investigation showed cerebral hemorrhagic foci secondary to leukostasis. He received leukoreduction apheresis and he was started on induction chemotherapy with daunorubicin and cytarabine. On the ninth day of induction chemotherapy, it was noted that he developed worsening neurological findings. Investigations showed significant elevation in ammonia level and associated cerebral edema. Although hyperammonemia was mitigated, the patient's cerebral status worsened and he died 15 days after initial presentation. This case shows that critical hyperammonemia can occur quickly after chemotherapy induction and that strategies for preventing a rise in plasma ammonia are necessary.