A Delicate Balance: A Case of Disseminated Intravascular Coagulation in Acute Promyelocytic Leukemia

Acute promyelocytic leukemia (APML) is a subtype of acute myeloid leukemia classically associated with a translocation between chromosomes 15 and 17. Advances in therapy, particularly the introduction of all-trans retinoic acid (ATRA), have significantly improved outcomes, with most patients achieving complete remission. Despite this, APML carries a high risk for complications such as disseminated intravascular coagulation (DIC) and hyperfibrinolysis, which can result in life-threatening hemorrhage. Below, we present a case of newly diagnosed APML complicated by cerebellar hemorrhage, which ultimately proved fatal.

A 65-year-old male with a medical history of cardiovascular disease and prior tobacco use presented with one week of hemoptysis. Initial workup revealed an abnormal complete blood count with differential, notable for severe thrombocytopenia, neutropenia, and elevated blasts. A bone marrow biopsy and cytogenetic analysis were performed, which confirmed a diagnosis of acute promyelocytic leukemia (APML). Treatment was initiated with ATRA and arsenic trioxide. Platelet and fibrinogen levels were closely monitored, with targets of maintaining platelets above 20,000/µL and fibrinogen above 150 mg/dL.

Several days into treatment, the patient reported a severe headache associated with nausea and vomiting. His neurologic exam was limited, as the patient was unable to follow commands and developed loss of bladder control. Emergent brain imaging revealed a large, acute cerebellar hemorrhage causing mass effect on the fourth ventricle, along with intraventricular hemorrhage in the third and lateral ventricles. The neurosurgery team evaluated the patient for possible evacuation of the hemorrhage. Additionally, he was given multiple units of platelets and fresh frozen plasma to achieve platelet levels greater than 100,000/µL and fibrinogen levels greater than 200 mg/dL. Despite aggressive blood product replacement, the patient continued to have coagulopathy and subsequently developed fixed, dilated pupils and became unresponsive to painful stimuli. Repeat imaging confirmed cerebellar tonsillar herniation and brain death.

This case underscores the potential for catastrophic hemorrhagic complications in APML, even with rapid diagnosis, prompt initiation of therapy, and appropriate supportive care. Despite close monitoring and efforts to maintain hemostatic targets, the patient developed a spontaneous cerebellar hemorrhage that ultimately led to the patient's demise.