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<p>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.</p>

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

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年5月27日

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

摘要

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.