Hydroxyurea-Associated Wunderlich Syndrome in Triple-Negative Myelofibrosis: A Case Report and Literature Review.

Abstract

Background: Wunderlich syndrome (WS) is a spontaneous, nontraumatic intrarenal or perirenal hemorrhage most commonly related to renal neoplasms, vascular disorders, infection, cyst rupture, or anticoagulation. Clinical severity ranges from self-limited bleeding to hemorrhagic shock.

Case: We report a 58-year-old woman with triple-negative myelofibrosis (MF) who was started on hydroxyurea (HU) 500 mg/day and low-dose acetylsalicylic acid (ASA). On Day 60, she presented with bilateral perirenal and intrarenal hemorrhage confirmed by contrast-enhanced abdominal computed tomography. ASA was discontinued and bleeding ceased spontaneously. One month later, she represented with recurrent bilateral hemorrhage without ASA exposure and marked neutrophilic leukocytosis. Concomitant purpuric skin lesions, positive antinuclear antibodies (ANA, 1:160), and low C3 levels raised suspicion for a HU-associated vasculitic process; HU was discontinued. Coagulation parameters at the time of bleeding (INR: 1.2, aPTT: 26.4 s, fibrinogen: 348 mg/dL, and D-dimer: 0.6 μg/mL) were not consistent with disseminated intravascular coagulation. Despite supportive management, the patient's condition deteriorated and she died shortly thereafter. The exact cause of death could not be definitively determined.

Conclusion: To our knowledge, this is the first reported case of WS in a patient with MF receiving HU therapy. Clinicians should consider WS in HU-treated patients presenting with acute flank pain or hematuria and evaluate for potential vasculitic features.