内科学-贫血总论谢彦晖课件.ppt

1、 Hematology Department,Huashan Hospital,Fudan University,Shanghai XieYan-HuiHemoglobin(Hb):male(adult)120g/L female(adult)110g/L female(gestation)100g/LRed cell count male4.5x1012 /L female4.0 x1012 /LHemocrit(HCT)male0.42 female0.37 female(gestation)0.30 B.Physical findings and complaintsa.Pale muc

2、ous membranes and skinsb.Weakness,loss of stamina,and exercise intolerance,Hypersensitivity to cold,fever.c.Tachycardia and polypnea,Heart murmur.anemia associated cardiac disease:Hb100 32 32-35 megaloblastic anemia MDSNormo 80-100 26-32 32-35 aplastic anemia,blood lost,hemolytic anemiaMicro 80 26 3

3、2 iron deficiency anemia sideroblastic anemia thalassemia B.Bone marrow response1.Regenerativea.Bone marrow actively responds by increasing its production of RBCs.b.Findings:(1)Polychromasia.(2)Reticulocytosisc.The presence of regeneration suggests an extramarrow cause.(1)Blood loss(2)Erythrocyte de

4、struction(hemolysis)d.Bone marrow examination would reveal erythropoietic hyperplasia.2.Non-Regenerativea.Inadequate bone marrow response because of a bone marrow disorder.b.Polychromasia and reticulocytosis are absent.C.Pathophysiologic mechanism1.Blood losshemorrhagic anemia.2.Accelerated erythroc

5、yte destructionhemolytic anemia.3.Reduced or defective erythropoiesis ANEMIA FROM ACCELERATED ERYTHROCYTE DESTRUCTION(HEMOLYTIC ANEMIA)A.Clinical findings1.Clinical signs of hemorrhage are absent.2.Jaundice may be seen in acute and severe cases.3.Hemoglobinuria and red plasma is seen if significant

6、intravascular hemolysis occursB.Laboratory findings1.Reticulocyte counts are higher in hemolytic anemias than externalhemorrhagic anemias 2.Plasma protein concentration is normal or increased.3.Neutrophilic leukocytosis and monocytosis may occur.4.Evidence of Hb degradation(hyperbilirubinemia,hemogl

7、obinuria).5.Abnormal erythrocyte morphology(Heinz bodies,erythrocytic parasites,spherocytes,or poikilocytes).I.Differentiation of the Causes of Hemolytic AnemiasA.Extravascular hemolysis1.Mechanismsa.Autoimmune Mediated-Antibody and/or C3 mediated(AIHA,infection,drug,immune system disorder)b.Decreas

8、ed erythrocyte deformability(a)Shistocytes of microangiopathic anemia(b)Spherocytes of immune-mediated anemia(c)Parasitized erythrocytes(d)Heinz body-containing cellsc.Reduced glycolysis and ATP content of the erythrocyte(PK deficiency)d.Increased macrophage activity (hypersplenism)e.Intravascular c

9、auses of hemolysis do not lyse all erythrocytes;some altered cells may remain that are removed by phagocytosis.2.Clinical and laboratory characteristics of phagocytic(extravascular)hemolysis.a.Usually chronic with insidious onset.b.A regenerative response.c.Hemoglobinemia and hemoglobinuria are abse

10、nt.d.Hyperbilirubinemiae.Neutrophilia,monocytosis,and thrombocytosisf.Splenomegaly.h.Low-grade extravascular hemolysis occurs in many anemias that are primarily nonhemolytic(e.g.,anemia of chronic renal disease,iron-deficiency anemia).Referred to as the“hemolytic component”of other types of anemiaB.

11、Intravascular hemolysisErythrocytes are destroyed within the circulation,releasing hemoglobin into the plasma where it is either removed by the liver or excreted by the kidneys.1.Mechanisms:The erythrocyte membrane must be significantly disrupted to allow escape of the Hb molecule into the plasma.Mo

12、st of the mechanisms of intravascular hemolysis are extrinsic or extracorpuscular defects the erythrocyte is initially normal.a.Complement-mediated lysis.(neonatal isoerythrolysis and transfusion reactions,PNH)b.Physical injury(Traumatic,microangio-pathic anemia,DIC,Coagulation,Vasculitis)c.Oxidativ

13、e injury(Heinz body,methemo-globin)d.Osmotic lysis(hypotonic intravenous fluids)e.Other membrane alterations.(1)Castor beansricin.Causes direct lysis(2)Snake venoms(3)Bacterial toxins(4)Parasites(Babesia)2.Clinical and laboratory characteristics of intravcascular hemolytic anemia.a.Most cases presen

14、t as peracute or acute episodes.b.History may reveal exposure to causative drugs or plants,recent transfusion of blood,or recent ingestion of colostrum.c.A regenerative response occurs,but it may not be evident in early stages.d.Hemoglobinemia is the principal feature of intravascular hemolysis.(1)R

15、ed discoloration of plasma(2)Increased MCHCe.Hemoglobinuria f.Hemosiderinuriag.Hyperbilirubinemiah.Additional laboratory findings may include schistocytes,keratocytes,Heinz bodies,erythrocytic parasites,positive Coombs test.ANEMIA FROM REDUCED OR DEFECTIVE ERYTHROPOIESIS reduced or defective erythro

16、poisis long or onset insidious clinic courseI.General considerations.A.Mechanisms:1.Precursor cells Nutrients(iron and B vitamins)Stimulation(erythropoietin)2.Bone marrow failure(intramarrow disease and extramarrow causes)3.Bone marrow failure may be selective for the erythroid series or may also af

17、fect the other cell lines.B.Bone marrow response1.When the number of precursor cells or erythropoietic stimulation is inadequate,the erythroid marrow is hypocellular.2.Maturation abnormalities which characterize the nutritional deficiencies,are associated with a Hypercellular marrow and ineffective

18、erythropoiesis.3.All degrees of bone marrow failure can occur,from complete aplasia to a suboptimal response of the erythroid marrow following hemorrhage or hemolysis.II.Differentiation of anemias caused by reduced or defective erythropoiesis.erythrocyte morphology,u blood neutrophil u platelet numb

19、ersu bone marrow cellularity.A.Normocytic,normochromic anemia;normal or increased neutrophil and platelet numbers;increased M/E ratio caused by hypocellular erythroid marrow.1.Anemia of erythropoietin lack.a.Chronic renal disease.Anemia proportional to severity of the uremia.b.Endocrinopathies (1)Cu

20、shings (2)Hypoandrogenism (3)Hypopituitarism 2.Anemia of chronic disorders(ACD)a.Occurs in chronic infectious,inflammatory,or neoplastic disorders.b.Cytokines involved with the inflammatory process initiate the anemia.c.Erythrocyte life span reducedd.Laboratory findings include:(1)Low serum iron (2)

21、Low total iron binding capacity (3)Increased bone marrow macrophage iron (4)Mild-moderate anemia that is usually nonprogressive 3.Pure red cell aplasia a.Characterized by a selective loss of erythroid precursors in the bone marrow.b.Thought to be immune mediated.4.Unknown mechanisms a.Liver disease

22、b.Vitamin E deficiency B.Normocytic,normochromic anemia;neutropenia and/or thrombocytopenia;M/E ratio is difficult to determine because of hypocellularity.1.Aplastic anemia a.pancytopenia.b.shorter life spans of the cells.c.Causes (1)Drugs,chemicals,plants (2)Irradiation (3)Cytotoxic T cells or anti

23、body (4)Infectious agents 2.Myelophthisic anemia a.The bone marrow is physically replaced by an abnormal proliferation of cells.(1)Myeloproliferative disordersleukemias (2)Myelofibrosis (3)Osteosclerosis (4)Diffuse granulomatous osteomyelitis (5)Metastatic cancer 3.Anemia caused by infectious agents

24、 a.Ehrlichiosis(埃里西提病）埃里西提病）b.FeLV（猫白血病病毒）（猫白血病病毒）C.Microcytic,hypochromic anemia;variable neutrophil and platelet number;usually a hypercellular marrow with a variable M/E ratio.1.Iron deficiency a.Chronic hemorrhage b.Dietary deficiency,especially in young milk-fed c.Ineffective erythropoiesis ear

25、ly;d.Laboratory findings:(1)Low serum iron (2)Variable iron-binding capacity (3)Microcytosis (4)Hypochromasia (5)Poikilocytes (6)Hypercellular bone marrow 2.Pyridoxine deficiency.This vitamin is a cofactor in heme synthesis and a deficiency leads to a failure to utilize iron.3.Copper deficiency.Copp

26、er-containing ceruloplasmin is important in iron absorption and transfer between gut,macrophages,and transferrin.D.Macrocytic,normochromic anemia;variable neutrophil and platelet number;M/E ratio usually low because of hypercellular erythroid marrow.1.Vitamin B12 and folic acid deficiency.2.Erythemi

27、c myelosis or erythroleukemia.3.FeLV infection.Treatment principle of anemia(1)causes treatment(2)supporting-trnsfusion(3)giving nutrition elements for hematopoiesis such iron agent,vitamine B12,folate acid(4)immunosuppression agent such glucocorticoid,antithymocyte globulin,cyclosporine A (5)auto-or allogeneic hematopoietic stem cell transplantation(6)hematopoietic growth hormone(EPO)or stimulating factor(androgen)(7)splenectomy写在最后写在最后成功的基础在于好的学习习惯成功的基础在于好的学习习惯The foundation of success lies in good habits55谢谢大家荣幸这一路，与你同行ItS An Honor To Walk With You All The Way讲师：XXXXXX XX年XX月XX日