骨髓增生性疾病课件_3.pptVIP

骨髓增生性疾病 Definition = Chronic Myeloproliferative Disorders (CMPD) = Myeloproliferative Neoplasm (MPN) Clonal haemopoiesis stem cell disorders Characteristics Proliferation (relatively normal maturation effective) in BM of 1 or More of the myeloid (eg. Granulocytic, Erythroid, Megakaryocytic) lineages Results in ↑ Numbers of Granulocytes, Red cells, Platelets (in Peripheral blood) 特点 都属骨髓干细胞病变，涉及四种类型的骨髓细胞（粒、红、巨核、纤维细胞） 可互相转化/合并存在 可直接/通过某种过渡类型转变为ANLL 都存在髓外造血，并伴核蛋白代谢异常 REVISED WHO CLASSIFICATION(2008) OF MYELOID NEOPLASMS MPN Myeloid/lymphoid Neoplasms ass. With eosinophilia other MDS/MPN MDS AML WHO Classification of Chronic MPDs Chronic Myeloid Leukaemia (CML) Chronic Neutrophilic Leukaemia (CNL) Chronic Eosinophilic Leukaemia (CEL) Polycythaemia Vera (PV) Chronic Idiopathic Myelofibrosis (CIMF) Essential Thrombocythaemia (ET) Chronic Myeloproliferative Disease, Unclassified (CMPD,U) Classification Non-Leukemic MPD (Benign) Polycythaemia Vera (PV) Essential Thrombocythaemia (ET) Myelofibrosis (MF) Leukemic MPD (Malignant) Chronic Myeloid Leukaemia (CML) Chronic Neutrophilic Leukaemia (CNL) Chronic Eosinophilic Leukaemia (CEL) MDS clonal stem cell disorders ? increased but ineffective hemopoiesis ? cytopenia CMPD clonal stem cell disorders ? increased hemopoiesis ? overproduction of one or more peripheral blood elements AML clonal stem cell disorders ? failure of maturation ? accumulation of myeloblasts in marrow ? pancytopenia, blastemia MDS M Stem cell A Ly My ??? R R Meg E GM O W ____________________________________________ BLOOD cytopenia CMPD M Stem cell ?? A Ly My ??? R R Meg E GM O W ____________________________________________ BLOOD AML M Stem cell A Ly My ??? R Progenitor cells ??? R O W