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DOI: 10.1055/a-2746-5438
The Interesting Case

Development of Essential Thrombocythemia Following Cessation of Hydroxyurea Therapy in an Elderly Patient with Carotid Arteritis and Recurrent Splenomegaly: A Diagnostic Dilemma

Entwicklung einer essentiellen Thrombozythämie nach Absetzen der Hydroxyharnstoff-Therapie bei einem älteren Patienten mit Karotisarteriitis und rezidivierender Splenomegalie: Ein diagnostisches Dilemma

Authors

  • Onur Aksoy 

    1   Radiology, Biruni University, Istanbul, Türkiye (Ringgold ID: RIN420479)

  • Rasım Cırpın

    1   Radiology, Biruni University, Istanbul, Türkiye (Ringgold ID: RIN420479)

  • Chousein Amet

    2   Pediatrics, Biruni University, Istanbul, Türkiye (Ringgold ID: RIN420479)

  • Erkan Demirci

    1   Radiology, Biruni University, Istanbul, Türkiye (Ringgold ID: RIN420479)

  • Birnur Yılmaz

    1   Radiology, Biruni University, Istanbul, Türkiye (Ringgold ID: RIN420479)
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Introduction

Persistent thrombocytosis and the potential for thrombohaemorrhagic consequences are hallmarks of essential thrombocythemia (ET), a myeloproliferative neoplasm [1]. Thrombotic and/or hemorrhagic episodes may exacerbate ET. Erythromelalgia, transient ischemic attack, myocardial infarction, deep vein thrombosis, or pulmonary embolism constitute a few of the most prevalent symptoms of small and medium artery thrombosis [2]. Patients with mildly symptomatic ET-associated aortic thrombosis may benefit from pharmacologic care alone, according to a few case studies; however, the drugs selected and the justifications for treatment vary widely [3] [4] [5] [6].

Large vessel vasculitis is a chronic inflammatory illness that affects the aorta wall and principal branches. Immune dysregulation is thought to be the cause of vessel wall inflammation. Systemic autoimmune diseases, such as giant cell arteritis, Takayasu arteritis, rheumatoid arthritis, Cogan syndrome, ankylosing spondylitis, and Behçet's disease, can all produce inflammatory large vessel vasculitis [3]. Malignancies are seldom the cause of recurrent large vessel vasculitis. Although the frequency of vasculitides associated with cancer ranges from 2.3% to 8.0%, the majority of vasculitides present as cutaneous [4] [5].

Herein, we present the rare co-occurrence of carotid arteritis and recurrent splenomegaly in a 69-year-old man with ET who had discontinued cytoreductive therapy. An elderly male patient was admitted to the department of radiology for unexplained splenomegaly and essential thrombocytosis. No significant pathology was observed in the bone marrow biopsy, and complaints of significant weight loss and abdominal pain developed recently. Therefore, the patient required additional imaging modalities, such as FDG PET-CT and neck Doppler ultrasound, to further investigate the underlying pathology in a detailed manner. FDG PET-CT revealed mild uptake along the left carotid artery, suspicious for large-vessel vasculitis, with minimally increased uptake in small lymph nodes and marked splenomegaly. Doppler ultrasound confirmed carotid arteritis. The patient was treated with steroids and planned for ruxolitinib, resulting in clinical improvement. This case report highlights the importance of comprehensive vascular assessment in patients with unexplained and persistent symptoms, as well as the inflammatory–thrombotic spectrum in essential thrombocytopenia.



Publication History

Received: 07 July 2025

Accepted after revision: 12 November 2025

Article published online:
25 November 2025

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