Cerebrovascular Infarcts in the Setting of Disseminated Intravasulcar Coagulation As Presenting Symptom in Acute Promyelocytic Leukemia

Introduction:

Patients with newly diagnosed Acute Promyelocytic Leukemia (APL) commonly present with disseminated intravascular coagulation (DIC) that can lead to hemorrhagic insult. Hematologists caring for APL patients understand that aggressive transfusion support with platelets, cryoprecipitate, and fresh frozen plasma must be initiated to correct the underlying DIC. Rarely, a patient with APL will present with arterial thrombosis in the setting of DIC and little is known about how to best manage these patients.

On review of the literature, five cases were described where patients with newly diagnosed APL presented with CNS arterial thrombosis; 3 patients are alive with neurologic deficit and 2 are dead. (Table 1).

We have experience with three patients diagnosed with APL who presented with cerebrovascular infarct. We report our experience to heighten awareness of this unusual presentation of this rare type of leukemia.

Methods:

Chart review was conducted with IRB approval on three patients with known diagnosis of APL who presented to Emory University with signs and symptoms of stroke who were found to have ischemic cranial events. Demographics, past medical history, and treatment regimen for both infarction and APL were collected.

Case Series:

Patient 1- 21-year-old male who presented to an outlying hospital with expressive aphasia. MRI scan showed large right temporoparietal infarction. Labs showed WBC 21.6, platelets 9,000, low fibrinogen, elevated PT and elevated d-dimer. High-risk APL was confirmed and ATRA and IV dexamethasone were started. Heparin drip was initiated to decrease risk of further ischemic insult. Worsening neurologic symptoms 4 days later led to a repeat MRI scan, which showed progressive right sided infarct and a new infarct in the left distal territory of the middle cerebral territory and expired shortly thereafter. Images 1 and 2 show infarcts on day 2 and 6 of admission.

Patient 2- 55-year-old female with presented with dysarthria and altered mental status. CT head showed left middle cerebral infarction and was immediately given tPA. Her admission labs showed WBC 37.6, platelets 48,000, PT 19, aPTT 35.3, Fibrinogen 67 and a diagnosis of high risk APL was established. She was induced with ATRA, Idarubicin and ATO and discharged in hematologic remission with no neurologic deficit.

Patient 3- 59 year old female, Jehovah's Witness, with multiple comorbidities presented with shortness of breath. Influenza A and PE were diagnosed and oseltamivir and warfarin was initiated. New left arm tingling prompted a brain MRI scan that showed multifocal bilateral acute infarcts. She was discharged with anticoagulation but readmitted 5 days later with a WBC of 38.4 and platelets 19,000. CT head showed progression of all the infarcts. APL was diagnosed and ATRA and ATO was started but she refused transfusions and died of neurologic deterioration.

Conclusions:

Our case series review is consistent with the previous literature. The mortality in patients with APL presenting with CNS arterial thrombosis is extremely high. Since it is a rare occurrence in a rare malignancy, not enough is known on the best management strategy. More dedicated observation and research is warranted on this topic to improve outcomes in this otherwise very curable malignancy.

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