[병리학] JAK Inhibition with Ruxolitinib versus Best Available Therapy for Myelofibrosis

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[병리학] JAK Inhibition with Ruxolitinib versus Best Available Therapy for Myelofibrosis에 대한 자료입니다.
본문내용
Myelofibrosis
Myeloproliferative neoplasm(MPN)
조혈모세포의 비정상적인 증식으로 인한 질환군, 정상적인 성숙과정을 보이는 효율적 조혈로 말초혈액에서 혈구의 수적증가를 초래한다.
대부분 massive splenomegaly 동반함

Polycytehmia : 정상적인 적혈구 생산 조절기전(Physiologid stimuli)과는 무관하게 주로 적혈구 생산이 증가되는 MPN, JAK2 mutation 동반
Primary myelofibrosis : 주로 megakaryocytes와 granulocytes가 증식되는 MPN으로 골수의 reactive fibrosis와 골수외조혈(extramedullary hematopoiesis)이 특징인 질환
Essenstial Thrombocythemia : 주로 megakaryocytes가 지속적인 thrombocytosis large,mature megakaryocytes의 증가가 특징인 질환