Essential Thrombocytosis: Digital Gangrene as First Manifestation

مقدمه/ بیان مساله : Essential thrombocythemia (ET) is a rare chronic myeloproliferative hematologic disorder, leading to an elevated platelet count. Two thirds of patients are asymptomatic during their lifetime, while others may experience symptoms like redness, congestion, and erythromelalgia after long symptom-free intervals.Significant symptoms, such as severe acrocyanosis and even peripheral gangrene, can be treated with a single dose of aspirin.معرفی بیمار : We present a rare instance of a ۵۵-year-old female who, despite receiving aspirin and losartan treatment, eventually developed digital gangrene. In further work-ups, she had an elevated platelet count and a positive JAK ۲ mutation. Her platelet count was reduced throughout treatment with aspirin, hydroxyurea, and heparin, which was followed by the necrotic tip of her index finger being surgically debrided. Daily aspirin consumption withstanding, this case developed the severe form of ET. In addition, while thrombocytosis predisposes patients to thrombotic complications in theory, there is little evidence to support a correlation between absolute platelet count and thrombosis. بحث و نتیجه گیری :This report made us aware that the initial symptoms of ET, an uncommon condition, could be this severe. Since the patient’s first manifestation of ET was arterial acral ischemia, this case suggests that thrombocytosis should be screened for in patients presented with arterial ischemia.

کلیدواژه ها:

Essential thrombocythemia ، Digital gangrene ، Platlet count