AML-173 Pseudotumor Cerebri (PTC) in Patients With Acute Leukemia (AL)

Context: Pseudotumor cerebri (PTC) is characterized by headache, vision changes, and fundus papilledema. Primary PTC is often seen in obese women (childbearing age) and secondary with medications such as all-trans retinoic acid (ATRA). Objective: We analyze clinical presentation, and outcomes of PTC in patients with acute leukemia (AL) treated at a general hospital. Design: Single-center retrospective study. Setting: We reviewed cases of AL patients with PTC at Memorial Hermann Hospital, Texas Medical Center between January 2013 and April 2022. Patients or Other Participants: Three (2.3%) of 127 patients (AML=85, APL=29, ALL= 13) had PTC. Interventions: Fit non-APL patients received induction chemotherapy (IC) with purine analogs-cytarabine-anthracycline. Unfit patients received a hypomethylating agent and venetoclax. APL patients received IC with arsenic trioxide and ATRA. Patients suspected of having APL were started on ATRA, and discontinued with a negative PML-RARA FISH test. Main Outcome Measures: We describe the incidence, risk factors, clinical presentation, and outcomes of AL patients with PTC. Results: Mean age was 42 years. Mean BMI was 42. All were white women. Two had AML, and one Philadelphia chromosome-positive ALL. AML patients received 3 doses of ATRA discontinued after negative PML-RARA FISH. One AML patient had PTC at presentation. The other developed PTC 3 months later. One AML patient had PTC 30 years before being treated for 6 months with acetazolamide; PTC recurred 3 months after her diagnosis. All patients had headaches, blurry vision, and papilledema with brain MRI showing optic sheath distension, globe flattening, and papilla cupping, all features suggestive of PTC. In two, the opening lumbar puncture pressures were elevated at 30 and 35 cm H2O. Cerebrospinal fluid cytology and flow cytometry were negative for leukemia. Two patients (AML=1, ALL=1) received intrathecal (IT) chemotherapy for CNS prophylaxis. All patient's symptoms improved with acetazolamide. LP done for IT chemotherapy helped to alleviate symptoms. No patient with APL (29 patients) developed PTC during treatment with ATRA. Conclusions: PTC is a rare diagnosis that can complicate the clinical course of AL patients. In addition to ATRA-induced PTC, clinicians should consider primary PTC, seen most often in morbidly obese women of childbearing age.