본태성혈소판증가증

본태성혈소판증가증
다른 이름Essential thrombocythemia, Essential thrombocythaemia, essential thrombocytosis, primary thrombocytosis
진료과혈액학 위키데이터에서 편집하기

본태성혈소판증가증(essential thrombocytosis, essential thrombocythaemia, essential thrombocythemia, ET), 본태성혈소판혈증(本態性血小板血症), 특발성 혈소판 증가증(primary thrombocytosis), 진성고혈소판증은 골수 내 거핵 세포로 인한 혈소판의 과생성을 특징으로 하는 희귀한 만성 혈액 질환이다.[1] 급성 골수성 백혈병이나 골수섬유증으로 발전할 수 있다.[1] 4가지 골수증식성질환 가운데 하나이다.[1]

증상

가장 흔한 증상으로는 출혈, 혈전증, 두통, 메스꺼움, 구토, 복통, 시각 장애, 어지러움, 실신, 손발의 저림이 있다. 백혈구 증가증, 빈혈, 비종이 가장 흔한 징표이다.[2][3][4]

병인

비정상 거핵세포에서 탄생한 혈소판들이 활성화되어 혈소판의 수를 증가시켜 혈전증의 가능성에 기여한다.[5]

치료

본태성혈소판증가증이 있다고 해서 반드시 치료가 필요한 것은 아니다.[6][7][8]

히드록시카바마이드(hydroxycarbamide), 인터페론α, 아나그렐리드가 혈소판의 수를 낮출 수 있다.

각주

  1. ↑ 가 나 다 Beer, PA; Green, AR (2009). “Pathogenesis and management of essential thrombocythemia” (PDF). 《Hematology / the Education Program of the American Society of Hematology. American Society of Hematology. Education Program》: 621–8. doi:10.1182/asheducation-2009.1.621. PMID 20008247. 
  2. ↑ Fu, R; Zhang, L; Yang, R (November 2013). “Paediatric essential thrombocythaemia: clinical and molecular features, diagnosis and treatment”. 《British Journal of Haematology》 163 (3): 295–302. doi:10.1111/bjh.12530. PMID 24032343. 
  3. ↑ Frewin, R; Dowson, A (October 2012). “Headache in essential thrombocythaemia.” (PDF). 《International Journal of Clinical Practice》 66 (10): 976–83. doi:10.1111/j.1742-1241.2012.02986.x. PMC 3469735. PMID 22889110. 
  4. ↑ Tefferi, A (March 2011). “Annual Clinical Updates in Hematological Malignancies: a continuing medical education series: polycythemia vera and essential thrombocythemia: 2011 update on diagnosis, risk-stratification, and management.”. 《American Journal of Hematology》 86 (3): 292–301. doi:10.1002/ajh.21946. PMID 21351120. 
  5. ↑ Vannucchi, AM (June 2010). “Insights into the pathogenesis and management of thrombosis in polycythemia vera and essential thrombocythemia”. 《Internal and Emergency Medicine》 5 (3): 177–84. doi:10.1007/s11739-009-0319-3. PMID 19789961. 
  6. ↑ Cervantes, F (2011). “Management of essential thrombocythemia.” (PDF). 《Hematology / the Education Program of the American ĺSociety of Hematology》 2011: 215–21. doi:10.1182/asheducation-2011.1.215. PMID 22160037. 
  7. ↑ Birgegård, G (July 2013). “Pharmacological management of essential thrombocythemia.”. 《Expert Opinion on Pharmacotherapy》 14 (10): 1295–306. doi:10.1517/14656566.2013.797408. PMID 23668666. 
  8. ↑ Tefferi, A; Barbui, T (August 2013). “Personalized management of essential thrombocythemia-application of recent evidence to clinical practice.” (PDF). 《Leukemia》 27 (8): 1617–20. doi:10.1038/leu.2013.99. PMC 3740400. PMID 23558521. 

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