Work

Management of pseudotumor cerebri syndrome in acute promyelocytic leukemia: use of a lumboperitoneal shunt to facilitate continued ATRA therapy - a case report

Year: 2026

Type: article

Abstract: Acute promyelocytic leukemia (APL) is associated with high early mortality, primarily due to hemorrhagic complications. Although treatment with all-trans retinoic acid (ATRA) and arsenic trioxide (ATO) has dramatically improved outcomes, ATRA may induce severe adverse effects such as pseudotumor cerebri syndrome (PTCS). We report a 19-year-old female with high-risk APL who developed recurrent PTCS during induction and consolidation therapy with ATRA. Treatment with acetazolamide and reintroduction of a lower dosages proved insufficient, and repeated lumbar punctures were contraindicated due to significant procedural anxiety and patient distress. A lumboperitoneal (LP) shunt was placed, keeping intracranial pressure stable and enabling uninterrupted ATRA administration and successful completion of consolidation therapy. To our knowledge, this is the first documented use of an LP shunt to manage refractory PTCS in an APL patient. The case highlights cerebrospinal fluid diversion as a potential strategy to sustain life-saving ATRA therapy in patients with recurrent PTCS unresponsive to conservative measures.

Source: Annals of Hematology

DOI: 10.1007/s00277-026-07078-x

Authors: Sara G. Creemers, Gizem Yildirim, Clemens M. F. Dirven, Nick Wlazlo

Institutions: Erasmus MC, Erasmus MC Cancer Institute

Language: English

Topic: Retinoids in leukemia and cellular processes

Subfield: Molecular Biology

Field: Biochemistry, Genetics and Molecular Biology

Domain: Life Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $4,590