內(nèi)分泌課件再生障礙性貧血英文課件aplasticanemia

1、內(nèi)分泌課件再生障礙性貧血英文課件aplasticanemia內(nèi)分泌課件再生障礙性貧血英文課件aplasticanemiaReview What is the function of bone marrowHematopoiesisReview What is the fuRed blood cells - transport oxygen and nutrientsWhite blood cells - fight infectionplateletshelp blood clotRed blood cells - transport Bone marrow haematopoiesis fa

2、ilure, HSC injuredPeripheral blood pancytopeniaTypical symptoms: anemia,infection,bleeding Direct and indirect pathophsiologic pathways causing A.AThe main characters of Aplastic AnemiaGeneral IntroductionBone marrow haematopoiesis faiClassification SevereModerateAbroadAcuteChronicHomeClassification

3、 SevereMoEpidemiologyIncidence China Annual incidence 7.4 per 100,000 6.01.4EpidemiologyIncidence 6.01.4DistributionEpidemiologyfemalemaleInfant70sDistributionEpidemiologyfemaleChemical factors: drug (anticarcinogen, chloramphenicol, sulfonamide, NSAIDs etc) chemicals (benzene, insecticides etc)Phys

4、ical factors radiation, X rayBiological factors viruses, severe bacterium, infectionOther pathogenic factorsUnknownEtiology Chemical factors:Etiology Pathogenesis Failure in hematopoietic stem or progenitor cells Defect in microenvironment of haematopoiesis Immuno-mediated bone marrow failure Geneti

5、c aspects: HLA associated susceptibility SeedsSoilHarmful InsectsPathogenesis Failure in hematoPresenting symptomsAcute AAChronic AAOnsetacute, short historyslowly onset,longer historyAnemiasevere, remarkablerelatively moderateBleedingheavy bleeding, multiorganhemorrhage moderate, skin and mucus Inf

6、ectionmore frequent,moderate, upper respiratory infectionsepsisPresenting symptomsAcute AAChrLaboratory findings Blood count（x109/L） granulocytes 0.5 platelets 20 reticulocytes 15 BM morphologyAcute AAChronic AAmultiplace hypocellularity lack of megakaryocyteslack of E,G increase in marrow fat incre

7、ased non-HCSimilar findings, focal area active haematopoiesisLaboratory findings Blood cBone marrow aspirationBone marrow aspirationNormal BM biopsyThis marrow is taken from a middle aged person, so it is about 50% cellular, with steatocytes admixed with the marrow elements.Normal BM biopsyThis marr

8、ow i Normal BM (high power magnification) Note the presence of megakaryocytes, erythroid islands, and granulocytic precursors. This marrow is taken from the posterior iliac crest in a middle aged person, so it is about 50% cellular, with steatocytes admixed with the marrow elements. Normal BM (high

9、power mAplastic anemia biopsyHematopoietic elements in this bone marrow biopsy are markedly reduced. Of course, RBC, platelets and granulocytes will often be diminished. Aplastic anemia biopsyMarrow FatAplastic anemia (HP)Marrow FatAplastic anemia (HP)BM morphology of Acute AARecover phase BM morpho

10、logy of Acute AARecovDiagnostic standard of AA (1987) DiagnosisDiagnosisSignBMPeri BloodexclusionDiagnostic standard of AA (19Diagnostic standard of AA (1987) Decreased peripheral blood count， absolute number of reticulocytes reduced Without megalospleniaDiagnosisDiagnostic standard of AA (19BM morp

11、hology DiagnosisModerate or severe hypocellularity found at least one aspiration (biopsy) site.Nonhaematopoietic cells increase in BM.BM morphology DiagnosisModerat Other diseases which may cause decreased blood count are carefully excluded，such as myelodysplastic syndrome(MDS), acute myelogenous le

12、ukemia(AML). Routine therapies for anemia fail to work.Diagnosis Other diseases which may caMyelodysplastic syndrome -dysmorphic featuresParoxysmal nocturnal haemoglobinuria (PNH)Hypoplastic Acute LeukemiaOthersDifferential Diagnosis - PancytopeniaMyelodysplastic syndromeDifferAn acquired haematopoi

13、etic stem cell defect with predominant haemolytic anaemia.A descriptive term for the clinical manifestation of haemolysis and haemoglobinuria manifest by dark coloured urine in the morning. PNH- Paroxysmal nocturnal haemoglobinuriaAn acquired haematopoietic steParoxysmal nocturnal haemoglobinuria.Hi

14、storically test was Hams test; showed red cell lysis by complement activation in acidified serum.Currently test for absent proteins on cell surface. CD55 and CD59Paroxysmal nocturnal haemoglobTreatmentSupportive CareGrowth HormonesImmune Suppressive TherapyHematopoietic Stem Cell TransplantationTrea

15、tmentSupportive CareGrowthRed cell transfusionPlatelet transfusionManagement of neutropenia private room face mask hand wash broad-spectrum antibiotics if feverSupportive CareRed cell transfusionSupportiveIndicated for patients 50 yearsPatients with no HLA matched sibling donorsAnti-Thymocyte Globul

16、in(ATG) or anti-lymphocyte globulin (ALG), cyclosporin, methylprednisoneBest results are for combination therapy.Response is slow, 4-12 weeks to see early improvementImmunosuppressive therapy Indicated for patients 50 yeResponse rates 60-70%Relapses are common and continued supportive care needed.Up

17、 to 50% of relapsed patients will respond to 2nd course of immunosuppressive therapyResponse rates 60-70%Age 55 years.Conditioning with Cyclophosphamide & antithymocyte globulin, with cyclosporin and methotrexate.Long term overall survival = 80-90%Chronic graft versus host disease (GVHD) remains a problem for 25-40% of patients.HSCT-HLA identical sibling HSCTAge 55 years.HSCT-HLA identic21 year manPresented with fatigue,pale,tirednessHb 11.0 WBC 2.6,neutrophils 1.1, platelets 45, MCV 104.B12/folate/ferritin were no