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Beyond the Surface: A Unique Presentation of Acute Promyelocytic Leukemia as Idiopathic Intracranial Hypertension: A
Amirhossein Mirhosseini1, Mirmassoud Shariat2, Mandana Ebrahimi3
1Department of Clinical Hematology and Oncology, Faculty of Medicine, Alborz University of Medical Sciences, Karaj, Iran.
Background:
Acute promyelocytic leukemia (APL) is a subtype of acute myeloid leukemia defined by a fusion gene transcript called PML::RARA. Pseudotumor cerebri (PTC) is characterized by elevated intracranial pressure (ICP) and presents with pulsatile tinnitus, headaches, and visual disturbances. Pseudotumor cerebri has been observed in some patients undergoing long-term ATRA therapy. While both APL and PTC have been individually described in the literature, their coexistence in a single patient, especially with PTC being the first clinical manifestation of APL, is an exceptionally rare occurrence, making this case particularly unique and warranting further investigation.
Case Presentation:
We present the case of a 30-year-old woman who initially presented with symptoms suggestive of PTC. The diagnosis of PTC was confirmed through a lumbar puncture. However, further investigations revealed that the patient had APL, which is typically sensitive to treatment with all-trans-retinoic acid (ATRA). To our knowledge, the association between APL and PTC as the first clinical manifestation of APL in adult patients has not been previously reported in the literature, further highlighting the exceptional rarity of this case.
Conclusions:
This case underscores the importance of considering underlying systemic diseases in patients presenting with PTC, as it may represent an exceptionally rare initial manifestation of a rare underlying malignancy such as APL. The coexistence of APL and PTC in this patient highlights the need for a comprehensive evaluation and management approach to promptly diagnose and treat coexisting conditions. Healthcare providers must be aware of this association and consider the possibility of APL when evaluating patients with PTC symptoms. Such awareness can prompt timely recognition and appropriate management, thereby improving patient outcomes and preventing potential delays in diagnosis and treatment.
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