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Reversible Posterior Leukoencephalopathy Syndrome Complicating Therapy of Hodgkin Disease in A Child with Familial Mediterranean Fever: A Case Report

Azza A G Tantawy, Fatma S E Ebeid, Nayera H k El Sherif


Introduction: Reversible posterior leukoencephalopathy syndrome (RPLS) is characterized clinically by headache, abnormalities of mental status and visual perception, and seizures. Despite its diverse causes, common precipitating factors are defined as abrupt elevations of blood pressure, renal decompensation, fluid retention, and immunosuppressive therapy.

Presentation of Case: We report a case of a child, aged five and half years old, having symptomatic FMF confirmed by genetic studies, who developed Hodgkin lymphoma while on colchicine therapy. The association between MEFV gene mutations, the gene responsible for familial Mediterranean fever (FMF), and hematolymphoid neoplasms has been recently suggested. Initiation of chemotherapy was complicated by development of RPLS. After initial good response to chemotherapy, the patient developed progressive Hodgkin disease with evidence of secondary amyloidosis despite regular colchicine therapy.

Conclusion: The causes of RPLS in our case could be multifactorial, that could stem from the abrupt elevation of blood pressure, high-dose chemotherapy, Hodgkin disease or the FMF.


Hodgkin disease; Familial Mediterranean fever; Reversible Posterior Leukoencephalopathy

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