内科贫血概述-陈春燕-15-12-16 课件

1、OVERVIEW OF ANEMIA仅限学术交流山东大学齐鲁医院 陈春燕 教授 ContentsAnemia Definition of Anemia Etiology and Pathogenesis Classification of Anemia Manifestations of Anemia Diagnosis of Anemia Treatment of AnemiaWHAT IS BLOOD?WHAT DOES BLOOD DO?VIA CIRCULATORY SYSTEM Bring oxygen & nutrients to cells Take away CO2 & oth

2、er wastes away from cells Carry WBC to the places where they are needed Bring hormones & antibodies to targeted organs or tissues or cells Red Blood Cells (Erythrocytes)Most abundant blood cellsContains the red pigment hemoglobin which binds and transports O2 and CO2Each RBC is a biconcave discEryth

3、rocytesWhy a biconcave disc?Provides a large surface area for O2 entry/exitEnables them to bend and flex when entering small capillariesRBCs lack a nucleus and most organelles.Instead they are simply membranous bags of hemoglobinHere, we have an RBC bending to fit thru a small capillaryAN = withoutE

4、MIA = blood ANEMIA = without blood1 DEFINITION OF ANEMIAANEMIA: Reduction below normal limits of the total red cell mass Reduction in hemoglobin concentration of blood（Hb） Reduction in numbers of red blood cells（RBC） Reduction below normal in volume of packed cells (hematocrit， Hct)外周血单位体积中血红蛋白浓度，红细

5、胞计数和(或)红细胞比容低于正常最低值者称为贫血。其中血红蛋白浓度最常用和可靠。DEFINITION OF ANEMIAGenderHb(g/L)RBC(1012/L)HctMale1204.50.42Female*1104.00.37 Female in pregnancy is defined as Hb100g/L. Hct 0.30Influential Factors of AnemiaAge SexAltitudeAlterations in plasma volume Blood concentration: diarrhrea, burning etc. Blood dilut

6、ion:congestive heart failure，etc.Blood loss2 Etiology and PathogenesisAlways bear in mind : Anemia itself is not a disease Anemia is a sign of many diseases Diagnosis of anemia is merely the first step to the process of searching for its causesEtiology and PathogenesisanemiaDecreased production Incr

7、eased destruction Blood loss Normal HematopoiesisFeatures of Hematopoietic Stem Cell Self-renewal: maintenance of stabilized pool size Multipotential differentiation: myeloid & lymphoid lineages Existance in BONE MARROW and PERIPHERAL BLOODErythropoiesisErythropoietin, EpoEtiology and PathogenesisRE

8、DUCED PRODUCTION OF RBC Marrow failure: reduction in stem cell Inherited: BMFS (Fanconi anemia etc.) Acquired: Aplastic anemia Ineffective erythropoiesis: MDS Marrow suppression: cytotoxic chemotherapiesEtiology and PathogenesisREDUCED PRODUCTION OF RBC Marrow infiltration: tumors, fibrosis etc. Dec

9、reased growth factors: renal failure (Epo) Abnormality of hematopoietic microenvironment Shortage in hemopoietic materials: folic acid, Vit.B12 deficiency ( megaloblastic) iron deficiency anemia (microcytic)2 Etiology and PathogenesisMicrocytic hypochromic RBCs from a case of IDAThe diameter of RBCs

10、 less than that of the nucleus of a normal small lymphocyte. And there are also some cells showing hypochromia,an area of central pallor that is larger than one third of the diameter of the red cells.2 Etiology and PathogenesisMacrocytic AnemiaRBCs with a diameter that considerably greater than that

11、 of the nucleus of a small lymphocyte.In additionthis smear shows ovalmacrocytes,defined as cells that are larger than normal and oval in shape(arrow).they are ofconsiderable being characteristic of megaloblastic anemia;They can be also be seen in dyserythropoiesis.Etiology and PathogenesisINCREASED

12、 DESTRUCTION OF RBCHemolytic anemia (shortened life-span of RBC) Intrinsic deficiencies: Inherited Membrane: spherocytosis,elliptocytosis, etc. Enzymes: G-6PD deficiency Hemoglubin: thalassemia,hemoglobinopathy Acquired Paroxysmal nocturnal hemoglobinuria（PNH）2 Etiology and PathogenesisHereditary sp

13、herocytosisA spherocyte is a red cell that lacks central pallor because of its spherical shape.In hereditary,there are usually cells in which the central pallor is reduced rather than absent,and they are intermediate in shape between a spherocyte and a discocyte,which is an erythrocyte with the norm

14、al shape of a biconcave disc.2 Etiology and PathogenesisTarget cells.A target cell is an erythrocyte with a hemoglobinizedarea in the middle of the normal area of central pallor.Teardrop poikilocytes.teardrop poikilocytes,or dacrocytes,are teardrop-shaped red cell;they are characteristic of primary

15、myelofibrosis but are also seen in megaloblastic anemia. Etiology and Pathogenesis Extrinsic factors: Immune related:antibody-mediated non Immune related: Physical: burning, etc. Chemical: toxics, medicine, etc. Biological: bacterials, viruses, viper venom etc. Blood Loss Acute Blood Loss: Traumatis

16、m or operative hemorrhage, Gynecological massive hemorrhage, Chronic Blood Loss: lesions in the Gastrointestinal tract or the Uterus. 2 Etiology and Pathogenesis3 Classification of Anemiacytometric classificationTYPESMCV(fl)MCH(pg)MCHC(%)Macrocytic1003231-35Normocytic80-10026-3231-35Microcyt-hypochr

17、802690-normal lower limitGrade2 (moderate)60 - 90Grade3 (severe)30 - 60Grade4 (very severe)30* life-threatening anemiaPhysiopathological ClassificationDecreased RBC ProductionIncreased RBC Destruction Marrow failure Ineffective hematopoiesis Marrow suppression: Marrow infiltration: Abnormal DNA synt

18、hesis Abnormal Hb synthesis Deregulation of erytheropoiesis Unknown or multiple mechanisms Intrinsic RBC abnormalities Extrinsic abnormalityBlood Loss Acute blood loss Chronic blood lossPhysiopathological ClassificationMarrow failure Aplastic anemia (AA) Fanconi anemia（FA） Pure red cell aplasia (PRC

19、A) Anemia due to renal failure Anemia due to endocrine disorders Congenital dyserythropoietic anemiaDecreased RBC ProductionPhysiopathological ClassificationIneffective hematopoiesis Myelodysplastic syndrome (MDS) Congenital dyserythropoietic anemia Megaloblastic anemiaDecreased RBC ProductionPhysio

20、pathological ClassificationMarrow infiltrationLeukemiacarcinoma metastasis to bone marrowmultiple myeloma myelofibrosis Decreased RBC ProductionPhysiopathological ClassificationAbnormal DNA Synthesis Vitamin B12 deficiency Folic acid deficiency Congenital or acquired defects in purine and pyrimidine

21、 metabolismDecreased RBC ProductionPhysiopathological ClassificationAbnormal Hb Synthesis Iron deficiency anemiaCongenital atransferrinemia Thalassemia and hemoglobinopathy Decreased RBC ProductionPhysiopathological ClassificationDeregulation of Erytheropoiesis Low oxygen affinity hemoglobinopathies

22、 High oxygen affinity hemoglobinopathies Decreased RBC ProductionPhysiopathological ClassificationDecreased RBC ProductionUnknown or Multiple Mechanisms Anemia of chronic disorders (ACD) Anemia due to nutritional deficiencies Sideroblastic anemiaPhysiopathological ClassificationIncreased RBC Destruc

23、tionIntrinsic abnormalities of RBC Membrane defects of RBC Hereditary spherocytosis Hereditary elliptocytosis Paroxysmal nocturnal hemaglobinuria Enzyme deficiency of RBC Glucose-6-phosphate dehydrogenase (G-6-PD) deficiency Pyruvate kinase(PK) deficiency Physiopathological ClassificationIncreased R

24、BC DestructionIntrinsic abnormalities of RBC Globin abnormality (Hempglobinopathy) Abnormal hemaglobinopathies Disturbance of globin synthesis (Thalassemia)Physiopathological ClassificationIncreased RBC DestructionExtrinsic abnormalities Antibody-mediated (Auto- or Allo-immune) Autoimmune hemolytic

25、anemia (AIHA) with warm-reacting antibody Cryopathic hemolytic disorders Drug-related antibody-mediated HA Alloimmune hemolytic anemia of newbornPhysiopathological ClassificationIncreased RBC DestructionExtrinsic abnormalities Physical or Mechanical Burning March hemaglobinuria Traumatic cardiac hem

26、olytic anemia Microangiopathic hemolytic anemia Chemical Toxic chemicals Medication or drugs Biological Pathogens (bacterials, viruses,parasites,etc.) Viper venom Hyperactivity of monocyte-macrophage system Hypersplenism Physiopathological ClassificationBlood Loss Acute Chronic4 Manifestations of An

27、emiaClinical picture of anemia consists of two components: underlying disease anemia itselfManifestations of anemia depend on the factors as follows: Lowered oxygen-carrying capacity Change in blood volume Velocity of developing the two above-mentioned factors Patients ability to compensate for anem

28、iaSeverity of anemia Hb70g/Lsymptoms begin Rapidity of anemia Abrupt loss of 20% of the circulating blood volume marked pallor, hypotension and tachycardia The loss of 50% severe shock even death. In contrast, the gradual loss (even Hb 3040g/L) of the circulating red cell mass in a patient with pern

29、icious anemia may occur without any symptoms at all.Manifestations of AnemiaWhy? Compensatory mechanismRed cell 2, 3 DPG (diphosphoglyceric acid） the oxyhemoglobin dissociation curve right shift increase the quantity of oxygen released in tissues Maximum elevation of RBC 2,3 DPG increases oxygen del

30、ivery only about 30 % , but this is a highly efficient form of compensation requiring no significant expenditure of energy.DPGCompensatory mechanismSelective contraction of blood vessels, so that more blood flows to critical organ Skin and renal blood flow reductionIncrease in cardiac outputSystemic

31、 Manifestations of AnemiaSkin and MucosaPALLOR-most frequent sign - Race - Pigmentation - Density and dilation of superficial blood vessels - Edema Observation of oral mucosa, conjunctiva, lip and nail-bed is more reliable Systemic Manifestations of AnemiaRespiratory & Circulation SystemsSymptomsSig

32、ns Tachycardia Dyspnea on exertion Systolic heart murmur Non-specific ECG changes ( T & ST) Hb 3mAnemic cardic diseaseSystemic Manifestations of AnemiaNeurologic & Muscular Systems Headache Dizziness Tinnitus Scotoma Faintness Lassitude Poor memory Weakness Fatigue Unable to concentrate Systemic Man

33、ifestations of AnemiaAlimentary SystemSymptomsSigns Loss of appetite nausea Fullness Abdominal discomfort Constipation or diarrhea Stomatitis Atrophy of papilla Dysphagia (rare)Systemic Manifestations of AnemiaUrogenital SystemSymptomsLab findings Polyuria Menorrhea MenostasisI Irregular mensis Loss

34、 of libido Proteinuria (mild)Systemic Manifestations of AnemiaMiscellaneous Low-grade fever (hypermetabolism) Jaundice (hemolytic) Hemoglobinuria( intravascular hemolysis) Hemoglobinemia5 Diagnosis of AnemiaHistory Onset Course Dietary pattern Medication Exposure Bleeding tendency Chronic disease Ch

35、ange in body weight Mensis Reproduction Tarry stool Dark urine Fever FamilyDiagnosis of AnemiaPhysical Examination Skin and mucosa (color, rash, ulceration) Spoon shaped nail Lymph nodes, spleen, liver Sternal tendernessDiagnosis of AnemiaLaboratory TestsScreening tests Complete cell count (CBC) ret

36、iculocyte Morphology of peripheral blood smear Bone marrow examination (if needed)Diagnostic tests (whenever indicated)The retic count (RC)RC:hyperplasticRC:hypoplasticBlood Smear Interpretation:NormalMicrocyteOvalTargetSpherocyteHeinz bodySchistocyteNucleated RBCPolychromaticTeardropNormal, RBCs. T

37、hey have a zone of central pallor (about 1/3 the size of the RBC). In the center of the field are a band neutrophil on the left and a segmented neutrophil on the right. The RBCs here are smaller than normal and have an increased zone of central pallor. This is indicative of a hypochromic (less hemog

38、lobin in each RBC) microcytic (smaller size of each RBC) anemia. There is also increased anisocytosis (variation in size) and poikilocytosis (variation in shape).Diagnosis of AnemiaSummary History taking is essential for approach to diagnosis Focused PE to find positive signs Judicious choosing of l

39、ab tests ensure pathologic classification LowMegaloblastic anemiaLow Hb=AnemiaMCVLowmicrocyticNormalnormocyticHighmacrocyticMeasure FerritinLowNormal/highIron def AnemiaAnemia ofchronic disease/Congenital Hb dis.Measure ReticshighlowMarrow failureRenal failureAnemia of chronic diseaseHemolytic anemi

40、a or blood lossMeasure B12 + folateNormal6 Treatment of AnemiaTreatment of the causes The purpose is the treatment of the underlying disease. Gastric canceranemiaTreatment of AnemiaSupportive CareTransfusion (Chronic anemia : 60 g/L；Abrupt loss of 30% of the circulating blood volume) whole blood (few indications) blood component (in most cases) Antibiotic therapy if indicatedTreatment of AnemiaSupplement of Nutrients or Factors Indicated in