中国医科大学附属第一医院：《内科学》课程教学资源（PPT课件讲稿，双语版）Anemias

Anemias 中国医科大学附属第一医院血液科李艳

Anemias 中国医科大学附属第一医院血液科 李艳

CUFS(造血干C、红系祖C) proerythroblast (原始RBC) Basophilic normoblast(早幼RBC) Polychromatic normoblast(中幼RBC) Orthochromatic normoblast(晚幼RBC)此阶段脱 核进入血循环 Reticulocyte(网织RBC) 经特殊染色见到丝状或网状结构,保留残余线粒体 和核糖,仍有继续合成Hb的能力(从早幼RBC开始) erythrocyte

CUF-S (造血干C、红系祖C) ↓ proerythroblast （原始RBC） ↓ Basophilic normoblast （ 早幼RBC） ↓ Polychromatic mormoblast（中幼RBC） ↓ Orthrochromatic normoblast （晚幼RBC）此阶段脱 核进入血循环 ↓ Reticulocyte （网织RBC） 经特殊染色见到丝状或网状结构, 保留残余线粒体 和核糖，仍有继续合成Hb的能力（从早幼RBC开始） ↓ erythrocyte

Definition a reduction below normal in the concentration of hemoglobin, the mass of red blood cells andor the hematocrit in the blood In men Hb 120g/L RBC≤4.5×1012L HcT<0.42 In women Hb≤110g/L RBC<40×1012L HCT<0.37

Definition • A reduction below normal in the concentration of hemoglobin , the mass of red blood cells and/or the hematocrit in the blood. In men Hb < 120g/L RBC <4.5×1012/L HCT <0.42 In women Hb < 110g/L RBC<4.0 ×1012/L HCT<0.37

Factors influencing Hb concentration Sex A ge Altitude Alterations in plasma volume

Factors influencing Hb concentration Sex Age Altitude Alterations in plasma volume

★ Classification Morphologic classification Type MCV(um) MCH(PG)MCHC(% Diagnosis Macrocytic 100 >32 32-35 Megaloblastic anemla Normocytic 80-100 26-32 32-35 AA, HA, Acute posthemorrhagic anemla Microcytic <80 <26 <32 DA Sideroblastic anemia Thalassem ias

★Classification Morphologic classification Type MCV(um) MCH(PG) MCHC(%) Diagnosis Macrocytic ＞100 ＞32 32-35 Megaloblastic anemia Normocytic 80-100 26-32 32-35 AA, HA, Acute posthemorragic anemia Microcytic ＜80 ＜26 ＜32 IDA, Sideroblastic anemia Thalassemias

>Kinetic classification ( Classified according to etiology and pathogenesis) Decreased erythropoiesis I Proliferation and differentiation abnormalities fsc HSC: AA. Fanconis anemia MDs Erythrocytic progenitors Pure red cell aplasia, Anemias caused by kidney failure and endocrine disorders

Kinetic classification (Classified according to etiology and pathogenesis) 一 、Decreased erythropoiesis 1 Proliferation and differentiation abnormalities of SC HSC: AA, Fanconis anemia, MDS Erythrocytic progenitors : Pure red cell aplasia, Anemias caused by kidney failure and endocrine disorders

2 Marrow infiltration leukemias carcinoma multiple myeloma myelofibrosis Malignant histocytosis

2 Marrow infiltration leukemias carcinoma multiple myeloma myelofibrosis Malignant histocytosis

3 Production and maturation blockages of differentiating cells DNA Synthesis blockage: Vit b12, falic acid difficiencies,嘌呤和嘧啶 metabolic defects->Megaloblastic anema Hb synthesis blockage Heme production defect IDA Heptoglobin production defect Thalassemias

3 Production and maturation blockages of differentiating cells DNA synthesis blockage : Vit B12, falic acid difficiencies, 嘌呤和嘧啶 metabolic defects→Megaloblastic anemia Hb synthesis blockage : Heme production defect : IDA Heptoglobin production defect : Thalassemias

4 Unknown reason or several echanisms Sideroblastic anemia Chronic disease anemias(chronic inflammation, infections,尿毒症 Hepatic disorders, neoplasm connective tissue disease. endocrine disorders)

4 Unknown reason or several mechanisms Sideroblastic anemia Chronic disease anemias (chronic inflummation, infections, 尿毒症, Hepatic disorders, neoplasm, connective tissue disease, endocrine disorders)

二、 Accelerated destruction of red cells 1) Endogenous (intra-erythrocyte defects) 1. Membrane defects of erythrocytes: Hereditary Hereditary spherocytosis (HS) Hereditas elliplocytosis (he) Acquired: PNH 2. Enzyme defects Glucose--6--Phosphate Dehydrogenase G6PD)deficiency Pyruvate kinase deficiency 3.Abnormal haptoglobin synthesis. Sickle cell anemia, Hemoglobinopathies Thalassemias

二、Accelerated destruction of red cells 1）Endogenous (intra-erythrocyte defects) 1.Membrane defects of erythrocytes : Hereditary :Hereditary spherocytosis (HS) Hereditasy elliplocytosis (HE) Acquired : PNH 2.Enzyme defects Glucose--6--Phosphate Dehydrogenase (G6PD) deficiency Pyruvate kinase deficiency 3.Abnormal haptogllbin synthesis : Sickle cell anemia, Hemoglobinopathies Thalassemias