AML-372 Acute Promyelocytic Leukemia: A Case of Pseudotumor Cerebri Syndrome During Treatment Consolidation

Abstract

Acute promyelocytic leukemia (APL) is a subtype of acute myeloid leukemia with distinctive morphological, clinical, and genetic features. Pseudotumor cerebri, also known as idiopathic intracranial hypertension, describes a neurologic syndrome characterized by headache, visual changes, papilledema without cerebral mass lesion, venous sinus thromboses, or obstructive hydrocephalus. Risk factors for the development of pseudotumor cerebri are obesity, women in the childbearing years, and hypervitaminosis A. The modified Dandy criteria are used for pseudotumor cerebri syndrome (PTCS) diagnosis. This complication has a high incidence in the pediatric population, particularly within 2-3 weeks of induction treatment for APL. Pseudotumor cerebri is a rare complication during consolidation therapy for APL. The reported incidence of "probable" PTCS is 1.7% in APL patients treated with ATRA. Case Report: We report here the case of a 20-year-old woman, obese with a BMI of 34.5 kg/m2, diagnosed with low-/intermediate-risk APL and treated with ATRA and ATO as induction treatment. After induction, she achieved MRD-negative complete morphological remission. During the first 2 weeks of the first cycle of consolidation-ATRA and ATO-the patient developed progressive diplopia and headaches without response to standard analgesic therapy. Additionally, she complained of nausea and vomiting. The ophthalmological exam with fundus examination revealed bilateral papilledema. The neurological exam was normal. There were no abnormal findings in the head CT scan or MRI of the brain. Thus, a diagnosis of "probable" pseudotumor cerebri was established. ATRA was discontinued and she received acetazolamide and dexamethasone with resolution of neurological symptoms within the next 7 days. Upon the next consolidation ATRA and ATO, the headaches resumed. The patient received all the ATRA consolidation cures as scheduled but with a dose reduction to 80% of the initial dose and in association with acetazolamide. She completed consolidation treatment and continues to be in molecular complete remission. Conclusions: This case report highlights the possibility of pseudotumor cerebri development in young adult patients with APL during consolidation therapy. The neurological symptoms were reversible, and the patient received all the ATRA consolidation. It is important to exclude other causes of intracranial hypertension including thromboses, infections, and disease involvement.