贫血总论

1,ANEMIA：General Aspects贫血总论,河北医科大学第二医院HEBEI Medical UniversitySecond Hospitol血液科Hematology department邢丽娜,2,内容 Contents,贫血的定义Definition贫血的分类classification临床表现 Clinical manifestation诊断 Diagnosis治疗 Treatment,3,简介 Introduction,贫血是一种临床常见的症状，由获得性或者先天性的原因造成的红细胞或其前体细胞异常的疾病引起。还有可能是其他非造血系统疾病引起。 Anemia is a commonly encountered clinical symptom that is caused by an acquired or hereditary abnormality of red blood cells (RBC) or its precursors, or may be a manifestation of an nonhematologic disorder,4,贫血是指人体外周血红细胞容量减少，即单位容积内血红蛋白（Hb）、红细胞（RBC）和或红细胞比积（HCT)低于正常参考值下限而由此带来的血液携氧能力的下降。 Anemia is defined as a decrease in the circulating RBC or hemoglobin ,haematocrit, and a corresponding decrease in the oxygen-carrying capacity of the blood.,定 义 Definition,5,正常值 Normal values of the hemogram:,RBC (1012/L) Hb (g/L) Hct (L/L）成年男性 (adult men） 4.0 5.5 120 160 0.400.50成年女性 (adult women) 3.5 5.0 110 150 0.370.48新生儿 (newborn) 6.0 7.0 170 200 孕 妇 (pregnant) 1001972 WHO criteria HCT: Haematocrit Hb: hemoglobin,6,定义 Definition,A decrease in any of these values (HCT, Hb, RBC count) is called anemia. (sea level) TEST WOMEN MEN HCT (%) 37 42 Hb (g/L) 110 120 RBC Count(1012/L) 4.0 4.5 我国贫血诊断标准（海平面地区）成年男性： Hb120g/L 成年女性（非妊娠) ：Hb110g/L 孕 妇： Hb100g/L,7,影响因素 Influential factor,贫血标准的正常值受多种因素影响：The mean normal value, low limits of the normal range depend on: 年龄：Agenewborns, infants 性别 : Gendererythropoietic effects of androgens 居住海拔高度:Altitude of residenceadults living at high altitudes 血浆容量:Plasmatic volumespregency，congestive heart failure， dehydration，acute hemorrhage ,8,9,生理性贫血Physiologic anemia,妊娠中、后期Pregnancy middle-late,3个月后的婴幼儿至15岁前的儿童Infants and young children from 3 mouths to 15 years old,老人Elderly,10,贫血分类 Anemia Classification,一、贫血的严重程度分级According to severity Classification 二、按红细胞形态分类 According to cell morphological classification三、按骨髓增生程度分类 According to Bone marrow proliferative degree四、根据病因和发病机理分类 According to etiology/pathogenesis,11,一、贫血的严重程度分级Classification according to severity,Degree Hb（g/L）Mild anemia 90120 Moderate anemia 6090Severe anemia 60Very Severe anemia 30,12,二 根据红细胞形态学分类Cell Morphological Classification,cell morphological type MCV（fl） MCHC (%)大细胞性贫血Macrocytic anemia 100 3235正常细胞性贫血Normocytic anemia 80100 3235小细胞低色素性贫血Microcytic hypochromic anemia 80 32,红细胞平均体积 MCV：mean corpuscular volume ；红细胞平均血红蛋白浓度 MCHC：mean cell hemoglobin concentration,13,14,大细胞性贫血 Macrocytic anemia,15,Macrocytic anemia 大细胞性贫血,16,正常细胞性贫血 Normocytic anemia,17,小细胞低色素性贫血Microcytic anemia,18,Microcytic hypochromic anemia小细胞低色素性贫血,19,三、按骨髓增生程度分类 According to Bone marrow proliferative degree,骨髓Bone marrow 贫血anemia 增生性贫血Hyperplasia IDA, hemolytic anemia, blood loss anemia增生不良性贫血Hypoplasia AA, PRCA成熟障碍性（红细胞无效生成）Dysmaturity Myelodysplastic syndrome(MDS) (ineffective erythropoiesis) Megaloblastic anemias,20,Hypoplasia,Hyperplasia,21,四、根据病因和发病机理分类 Classification according to etiology/pathogenesis,根据病因和发病机制的分类更能反映贫血的病理本质 The classification according to etiology/pathogenesis can represent the nature of anemia1. 红细胞生成减少 Deficient RBC production 2. 红细胞破坏增多 Increased RBC destruction 3. 失血性贫血 RBC Loss anemia,22,1.红细胞生成减少性贫血 Deficient RBC Production,（1）骨髓干祖细胞异常 BM stem cells abnormality 1.再生障碍性贫血（Aplastic anemia，AA） 2.纯红细胞再生障碍性贫血（Pure red cell anemia，PRCA） 3.骨髓增生异常综合症（Myelodysplastic syndrome，MDS） 4.先天性红细胞生成异常性贫血（Congential dyserythropoietic anemia，CDA） 5. 造血系统肿瘤 Hemotologic tumor eg AL,NHL,MM,23,1.红细胞生成减少性贫血 Deficient RBC Production,（2）造血调节异常所致贫血 Hematopoiesis modulation abnormality 1.骨髓基质受损导致贫血（Marrow stromal damage） 骨髓纤维化 (Myelofibrosis ,MF) 骨髓坏死（Bone marrow necrosis） 骨髓硬化症 （Myelosclerosis） 骨髓转移癌（Metastasis to bone marrow） 骨髓炎（Infectious or non-infectious osteomyelitis ）2.淋巴细胞功能亢进导致贫血 （lymphocyte hyperfunction ） 免疫相关性全血细胞减少（Immuno related pancytopenia，IRP） 3.造血调控因子水平异常(lack of erythropoietin production) 肾功能不全，垂体或甲状腺机能低下，肝病等慢性病贫血（ACD) Renal disease, Hypopituitarism ,Hypothyroidism, Live disease Anemia of chronic disease（ACD),24,1.红细胞生成减少性贫血 Deficient RBC Production,（3）造血原料不足或利用障碍 Deficiency of essential substances or Ultilization obstacle 1. 缺铁性贫血 Iron deficiency anemia 2.叶酸或维生素B12缺乏或利用障碍 Folic acid and/or Vit B12 deficiency Pernicious anemia,25,2. 红细胞破坏增多-溶血性贫血 Increased RBC destruction-hemolytic anemia,1.红细胞内在缺陷（RBC internal defect）(1)红细胞膜缺陷（RBC membrane defect） 获得性 acquired: PNH 先天性hereditary : hereditary spherocytosis(2) 酶缺陷 enzyme deficiency： G6PD deficiency (3) 血红蛋白异常Hb abnormality： hemoglobinopathy(4) 卟啉代谢异常porphyrin metabolism：porphyrinopathy2. 红细胞外在缺陷（RBC exopathic defect） 免疫性，化学性，生理性，物理性因素 immunologic, chemical, biological, or physical factors,26,3. 失血性贫血 RBC Loss anemia,Hemorrhage 急性失血Acute blood loss慢性失血Chronic blood loss 创伤失血Due to trauma组织损失导致的失血，例如肿瘤，溃疡病，结核病，应激性肠病（包括溃疡性结肠炎和克隆氏病Due to disorders: e.g.cancer, ulcers, tuberculosis, and irritable bowel syndrome (including ulcerative colitis and Crohns disease) 月经出血 Menstrual flow妇产科疾病导致失血（例如子宫内膜异位症，肌瘤）Gynecological disorders (e.g. endometriosis, fibroids) 寄生虫 Parasitism 钩虫病 Hookworms,27,Related factor;引起贫血的病因 cause of anemia贫血导致血液携氧能力的下降程度 Degree of decrease of oxygen carrying capacity 3. 贫血时血容量下降的程度 Degree of decrease of blood volume4. 发生贫血的速度Speed of anemia genesis5. 系统的代偿和耐受能力 Compensation and tolerance of anemia,临床表现 Clinical manifestation,28,1、神经系统 Nervous system：头痛、眩晕、萎靡、昏厥、失眠、多梦、耳鸣、眼花、记忆力减退、注意力不集中，感觉麻木，智力发育异常。headache、dizzy、dispirited、faint、insomnia、 dreamful sleep， ear noises、dim eyesight 、memory impairment、impaired concentration；acroanesthesia , intelligence development abnormality2、皮肤粘膜：苍白，粗糙，溃疡，黄染，反甲skin and mucosa：pallor；rough ；ulcer, stained yellow, Koilonychia,临床表现Clinical manifestation,29,30,5 . 呼吸和循环系统 Respiratory & cardiovascular system心悸 Palpitation呼吸困难 Dyspnea呼吸急促 Tachypnea 心动过速 Tachycardia血压升高 Increased artery blood pressure心脏扩大 Enlargement of heart心力衰竭 Even heart failure,31,4、消化系统：腹部饱胀，食欲减退，厌食，恶心,大便规律紊乱，便秘,吞咽异物感，镜面舌Digestive system：abdominal distention、 Decreased food appetite, anorexia, nausea, bowel disturbance, constipation, swallow foreign body sensation；,32,6、泌尿系统：胆红素尿，血红蛋白尿，含铁血黄素尿，急性肾功能衰竭Urinary system：bilirubinuria 、hemoglobinuria、urinary siderosis；ARF (acute renal failure )7、内分泌系统：内分泌功能异常Endocrine system： endocrine functional abnormality8、免疫系统：免疫功能低下，容易感染immune system： immunodeficiency ，vulnerable to infections,Clinical manifestation,33,9、血液系统 Hematological system：外周血：血细胞数量，形态和生化成分异常。可以合并血浆或血清成分的异常。造血组织可以合并肝脏，脾脏，淋巴结肿大。 peripheral blood: blood cell count, appearance and biochemical component；plasma ,serum components abnormal hematopoietic organ :liver , spleen, lymph node, Hepatosplenomegaly 10、生殖系统：在男女两性中性欲减退均多见,男性性征减弱，女性月经紊乱月经失调如月经过多或闭经 Genital system ： hyposexuality ,weaken male sexual characteristics ，paramenia ,menses disturbance，menorrhagia, menoschesis,临床表现Clinical manifestation,34,完整的诊断 Complete diagnosis是否有贫血 Anemia贫血的程度 Degree of anemia 贫血的分类 Classification of anemia 贫血的病因 Cause of anemia,诊断 Diagnosis,35,询问病史 History(inquisition):现病史（症状和持续时间）Present history(symptoms and their duration)既往史（是否有胃切除，痔疮，胃溃疡病史） Past history (Gastrectomy, haemorrhoids, peptic ulcer)3. 家族史（遗传背景）Family history(hereditary background)4. 月经和生育史 Menses and childbearing history5. 饮食习惯 Diet habit6. 接触的危险因素（例如化学药物和毒物）Exposure to risk factors(such as chemicals or toxins),诊断： 病史 Diagnosis: history,36,诊断：物理检查Diagnosis: Physical examination,苍白 （观察嘴唇，眼睑，结膜，甲床，舌乳头萎缩） Pale (lip, palpebral, conjunctiva, finger nail ，atrophy of tongue papillae )2.伴随体征 accompanying symtoms 溶血 hemolytic：jaundice hepatosplenomegaly 出血 bleeding：bleeding point，purpura，ecchymosis 浸润 infiltration ：lymphadenectasis， hepatosplenomegaly ,bone tenderness 感染 infection 营养不良 malnutrition：,37,外周血 Peripheral blood(PB