Digital Microvascular Occlusion in Essential Thrombocythemia: A Case Report and Review of Management Strategies

Abstract

Aim: This case report aims to address the apparent lack of documented instances of digital microvascular occlusion secondary to essential thrombocythemia (ET) in the southwestern region of Nigeria, while also providing a review of management strategies. Presentation of Case: This report describes the case of a 75-year-old patient exhibiting signs of erythromelalgia, characterized by dusky, congested, and hyerpigmented foot. The patient experienced vascular complications due to a delayed presentation at Redeemers Health Village, a multi-specialty tertiary academic hospital in western Nigeria. The patient was diagnosed with JAK2 negative ET based on a Doppler ultrasound that revealed atherosclerotic arterial stenosis. His diagnosis met four criteria outlined in the WHO guidelines, including a sustained platelet count greater than 450,000/µL. Given his background history of a thrombotic stroke, the ultimate goal of treatment was to reduce the risk of thrombosis or hemorrhage.  The patient underwent cytoreduction therapy, which successfully reduced his initial platelet count from 809,000/µL at presentation to 198,000/µL. Following this treatment, he underwent a below-the-knee amputation. Postoperative management included rehabilitation, ambulation, and the use of a below-the-knee prosthesis. Discussion: Essential thrombocythemia (ET) is often asymptomatic and is therefore frequently detected incidentally, particularly in facilities equipped to recognize it. The limited availability of specialized care in many community and general hospitals may contribute to the perceived rarity of ET. As illustrated in this case report, if left untreated for extended periods, ET can lead to increased morbidity and mortality due to conditions that predispose patients to microvascular disease. These risks can be minimized by implementing measures to reduce the time to treatment. Conclusion: Essential thrombocythemia may not be as uncommon as previously suggested. Patients with this condition are often asymptomatic and may remain unaware of their diagnosis until they experience a sudden thrombotic event. Identifying this condition through routine laboratory tests requires the expertise of a haematologist.The case points towards the significance of early intervention and intensive treatment since delay in presentation leads to critical complications needing significant surgery.