英文班貧血總論課件

1、Anemia- General aspectsDepartment of HematologyRui Jin HospitalAnemiaMost importantRelated to many diseasesDiagnosed properlyTreated properlyWhat is anemia?A reduction below normal in the- concentration of hemoglobin- red blood cells in the blood- hematocrit （紅細(xì)胞比容）The blood hemoglobin concentration

2、 often preferred- accuracy and reproducibility- value most indicative of the patho-physiologic consequences of anemiaRBC（1012L） HGB（g/L）HemotocritM 4.0-5.5 (400-550萬(wàn)mm3) 120-160 (12-16g/dl) 0.4-0.5 (40-50) F 3.5-5.0 (350-500萬(wàn)mm3) 110-150 (11-15g/dl) 0.37-0.45 (37-45) Normal range - adultConditions a

3、ssociated with a significant disproportion between the hematocrit and RBC?Spurious anemia (假性貧血)- relative increase in plasma volumehydremia of pregnancyoverhydration in oliguric renal failure or congestive heart failurechronic diseases and hypoalbuminemiacongestive splenomegalyrecumbency (斜臥)Normob

4、lastsPronormoblastRegulation of erythropoiesisHow many are produced?25 billion /24 hoursThe entering cells are reticulocytes which should be 1% of the total population of circulating erythrocytesErythrocytes last 120 days and are destroyed by the spleen Red cell production should equal red cell dest

5、ructionAnemia: EtiologiesProduction defectsBlood lossBlood destructionRBC loss without RBC DestructionHemorrhage- Due to trauma- Due to disorders: e.g. cancer, ulcers, tuberculosis, and irritable bowel syndrome (including ulcerative colitis and Crohns disease) Menstrual flowGynecological disorders (

6、e.g. endometriosis)Pregnancy, especially at gestationParasitism- HookwormsRBC DestructionIntrinsic Abnormalities ThalassemiaG6PDSickle Cell AnemiaHereditary SpherocytosisExtrinsic AbnormalitiesInfectionsMalaria (Plasmodiumm species)Mycoplasma (支原體）Disseminated Intravascular CoagulationLead poisoning

7、 Conditions Associated with Anemias Due to Reduced Erythrocyte ProductionAnemias due to decreased erythropoietin production Renal disease Endocrine deficiency (pituitary, adrenal, thyroid, testis) Starvation Hemoglobinopathy (reduced oxygen affinity) Anemias due to inadequate marrow response to eryt

8、hropoietin Deficiency state (iron, vitamin B12, folate) Anemia of chronic disease (inflammation, infection, or malignancy) Sideroblastic anemia Primary marrow disorders-pure red cell aplasia, myelodysplasia, myelofibrosis, leukemia metastasis to bone marrow, osteogenic sarcoma貧血的病理生理(pathophysiology

9、 of anemia) RBC的主要功能是輸送氧氣 正常血液一克Hb能攜帶1.34ml氧 貧血的病理生理基礎(chǔ)是血液攜氧能力減低，組織缺氧 機(jī)體對(duì)缺氧進(jìn)行代償和適應(yīng)機(jī)制如下貧血的病理生理心臟搏出量增加 貧血患者因RBC減少，血液粘度減低和選擇性的周圍血管擴(kuò)張，周圍血管阻力減低心率加速，循環(huán)速度加快為防止對(duì)機(jī)體供氧量減少，心臟排血量增加貧血的病理生理增加組織的灌注 ：貧血時(shí)血液供應(yīng)重新分配，供血減少區(qū)域?yàn)槠つw組織和腎臟，故皮膚蒼白，對(duì)缺氧敏感的心肌、腦和肌肉供血量增加 肺的代償機(jī)能 ：貧血時(shí)呼吸加快、加深、呼吸增強(qiáng)，但這并不能得到更多的氧，這可能是對(duì)組織缺氧的一種反應(yīng) 貧血的病理生理氧解離曲線右移

10、 在緩慢發(fā)生的貧血，RBC內(nèi)2、3二磷酸甘油酸（2、3DPG）的合成增加2、3DPG使Hb與氧的親和力減低促進(jìn)Hb O2解離曲線右移，使組織在氧分壓降低的情況下能攝取更多的氧 Clinical manifestationFirst symptom- decreased work tolerance- shortness of breath- palpitations- other signs of cardiorespiratory adjustments to anemia- at times, they feel fine, but their friends or family note

11、s the pallorThe manifestations depend onThe reduction in the oxygen-carrying capacity of the bloodThe degree of change in total blood volumeThe rate at which these two factors developedThe capacity of the cardiovascular and pulmonary systems to compensate for the anemiaThe associated manifestations

12、of the underlying disorderCardiorespiratory system Noticeable only after exertion or excitementDyspnea and awareness of vigorous or rapid heart action noted at restShortness of breath, tachycardia, dizziness or faintness, and extreme fatigueCongestive heart failure, angina pectoris, intermittent cla

13、udication （間歇性跛行）Cardiorespiratory systemLeft ventricular hypertrophy Heart murmurs- systolic in pulmonic areaElectrocardiographic changes- depression of the ST junction- U-shaped deformation of the S-T segment- inverted T wavesThe skinPallor most evident sign- dilation of the peripheral vessels- de

14、gree and nature of the pigmentation- nature and fluid content of the subcutaneous tissues- jaundice, cyanosisDetected most accurately in the mucous membranes of the mouth and pharynx, conjunctivae, lips, and the nail beds, palms of the handsTell anemia from the color of the skinA waxy, dead whitenes

15、s- acute blood lossA distinctly sallow （灰黃色）color- chronic anemiaA lemon-yellow pallor- pernicious anemia（惡性貧血）Marked pallor associated with petechiae or ecchymoses（瘀斑）- acute leukemiaNeuromuscular systemHeadacheVertigoTinnitus（耳鳴）FaintnessScotomata （盲點(diǎn)）Lack of mental concentrationDrowsinessRestless

16、nessMuscular weaknessGastrointestinal systemUnderlying diseases: ulcer, cancerGlossitis and atrophy of the papillae of the tonguePainful, ulcerative, and necrotic lesions in the mouth and pharynx (aplastic anemia, acute leukemia)Dysphagia (chronic iron-deficiency anemia)OthersRetinopathy: 20%- flame

17、-shaped hemorrhagesSlight proteinuria Fever of mild degreeHemolytic anemia- jaundice- hemoglobinemia, pain in the abdomen or back Evaluation and diagnosisMedical HistoryPhysical examinationLaboratory testsMorphologic featuresMedical historyThe duration of the symptoms and their onset (insidious/acut

18、e)Family history of jaundice, splenectomy, bleeding disorders, and hemoglobin-associated abnormalitiesOccupation, drugs, solvents and other chemicalsOthersStool habits: neoplasms of the colon and rectum, hemorrhoidsWomen: blood lost during menstruationChild or adolescent: growth rateDietary historyF

19、ever: infection, lymphoma, collagen disease, etc.Bruises, ecchymoses, and petechiaePhysical examinationIcterus 黃疸 (hemolytic)Sternal tenderness (leukemia)Lymph nodes enlargements (infection, lymphoma, leukemia, metastatic carcinoma)Cardiac murmurs (bacterial endocarditis)Liver, spleen, and kidneysMo

20、rphologic featureBlood smearBone marrow examinationClassificationImpaired erythrocyte production- hypoproliferativeiron-deficient erythropoiesiserythropoietic deficiencyhypoplastic anemiainfiltration- ineffectivemegaloblasticmicrocyticIncreased erythrocyte production- hemolytic anemiaAnemia?Producti

21、on?SurvivalDestruction?The key test is the .The reticulocyte countIncreased reticulocytes (greater than 2-3% or 100,000/mm3 total) are seen in blood loss and hemolytic processes, although up to 25% of hemolytic anemias will present with a normal reticulocyte count due to immune destruction of red ce

22、ll precursors. Retic counts are most helpful if extremely low (100 MCV 80-100MCV80Macrocytic NormocyticMicrocytic(大細(xì)胞性) (正常細(xì)胞性)(小細(xì)胞性)Macrocytic anemiasVitamin B12 or folate deficiencyAccelerated erythropoiesis- hemolytic anemia Erythroleukemia (紅白血病)Increased membrane surface- hepatic disease, obstr

23、uctive jaundiceRefractory anemias- myelodysplastic anemiasNormal, RBCs. They have a zone of central pallor about 1/3 the size of the RBC.). A few small fuzzy blue platelets are seen. In the center of the field are a band neutrophil on the left and a segmented neutrophil on the right. Note the hypers

24、egmented neurotrophil and also that the RBC are almost as large as the lymphocyte. Finally, note that there are fewer RBCs.Vitamin B12 deficiencyDietary deficiency (rare)Lack of intrinsic factor- pernicious anemia- gastric surgeryFunctionally abnormal intrinsic factorBiologic competition- small-bowe

25、l bacterial overgrowthDrug-induced vitamin B12 malabsorptionChronic disease of the pancreasZollinger-Ellision syndromeHemodialysisFolate deficiencyDietary deficiencyIncreased requirements- alcoholism and cirrhosis- pregnancy- infancyCongenital folate deficiencyExtensive intestinal resectionDiagnosis

26、Smear- Macrocytic (High MCV) RBCs, +/- hypersegmented neutrophils, +/- modest neutropeniaB12- Low serum B12 levelFolate- Serum folate level - can normalize with a single good mealNeurological presentation of B12 deficiencyParesthesias of fingers & toesReduced vibration sense (256-Hz)Ataxia 共計(jì)失調(diào) (Com

27、bined system disease)Perversion of taste & smell Optic atrophyDementia (癡呆), Memory Loss & Depression“Megaloblastic Madness” Paranoid SchizophreniaSpecific MRI abnormalitiesTreatmentB12 deficiency- B12 1 mg/month IM, or 1-2 mg/day poFolate deficiency- Improved diet, 5mg tabletsMonitor for a response

28、 to therapyPernicious Anemia monitor for GI cancersFoodB12 deficiencyMeatEggsFolate deficiencyGreen vegebablesNutsCerealFruitYeastMicrocytic anemiasDisorders of iron metabolism- iron-deficiency anemia- anemia of chronic disordersDisorders of globin synthesis- thalassemias- hemoglobin E and C disease

29、Sideroblastic anemiaThe RBCs here are smaller than normal and have an increased zone of central pallor. This is indicative of a hypochromic (less hemoglobin in each RBC) microcytic (smaller size of each RBC) anemia. There is also increased anisocytosis (variation in size) and poikilocytosis (variati

30、on in shape).Iron Deficiency Anemia vs. anemia of chronic disordersSmear- hypochromic and microcytic (low MCV) RBCs, usually not seen unless Hct 30%- platelet count is often elevatedFerritin: a measure of total body iron stores, but also an acute phase reactant- 15g/l = Fe deficiency- 150 g/l = Not

31、Fe deficiency - 15-150 g/l = Not conclusiveBone marrow- absent Fe stores Gold standard for IDAThalassemiasGenetic defect in hemoglobin synthesis- synthesis of one of the 2 globin chains ( or )- Imbalance of globin chain synthesis leads to depression of hemoglobin production and precipitation of exce

32、ss globin (toxic)- “Ineffective erythropoiesis”- Ranges in severity from asymptomatic to incompatible with life (hydrops fetalis)- Found in people of African, Asian, and Mediterranean heritageThalassemiasDiagnosis- Smear: microcytic/hypochromic- -thal will have an abnormal Hgb electrophoresis (HbA2,

33、 HbF)- The more severe -thal syndromes can have HbH inclusions in RBCs- Fe stores are usually elevatedTreatment- Mild: None- Severe: RBC transfusions + Fe chelation, Stem cell transplantsSideroblastic AnemiasHeterogenous grouping of anemias defined by presence of ringed sideroblasts in the BMEtiologies- He