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内容参考分析内科学aa.pptx

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Aplastic anemia Zheng Runhui , Dept. of Hematology, The 1st Affiliated Hospital, GMUCONTENTSINTRODUCTIONETIOLOGY /PATHOGENESISMANIFESTATIONSLABORATORY FINDINGSDIAGNOSIS DIFFERENTIAL TREATMENTAplastic anemia(AA)Definition AA , an unusual hematologic disease, is the paradigm of the human bone marrow failure syndromes. -marrow hypoplasia -peripheral pancytopeniaIncidence: 7.4 per million population East West Biphasic peak at 20-25 and 60 yCauses: Inherited (20%)Fanconi anemiaDyskeratosis congenitaCartilage hair hypoplasiaPearson syndromeAmegakaryocytic thrombocytopeniaShwachman-Diamond syndromeDubowitz syndromeDiamond-Blackfan syndromeFamilial aplastic anemiaCauses: Acquired (80%) Infections: hepatitis, EBV, HIV, parvovirusRadiation and chemicals such as benzeneDrugs: chloramphenicol, phenylbutazone ,goldPNH (paroxysmal nocturnal hemoglobinuria)Transfusional graft versus host diseaseLiver transplantationPregnancyPathogenesisDepletion of hemopoietic stem cellSuppression of proliferation and maturation Mechanism -Immune related -Cytotoxic T-cell, INF (Interferon ) γ, TNF(tumor necrosis factor )α↑PresentationAnemia: pallor, headache, palpitations, dyspnea, heart failureThrombocytopenia: mucosal and gingival bleeding or petechiaNeutropenia: infections, mouth and pharyngeal ulcerationsTestsPB, BM( multiple) smear, biopsy, cytochemistryChromosomeT cell number and functionOthers Virus Ab (hepatitis, herpes, rubella…) Coombs test (direct indirect) Vit B12 ANA (antinuclear antibody), C3, C4 Hb electrophoresis, sugar water test HLA typing, BM chromosomeBM aspiration and biopsyDIAGNOSIS OF AACriteria direct evidences exclusive evidencesSubtypes SAA (severe AA) VSAA (very severe AA) NSAA (not severe AA)CRITERIA OF AADIRECT EVIDENCES:PancytopeniaHypocellularity of BM 3 myeloid lineages obviously reduced in BMIncreased percentages and function of T lymphocytesNormal morphologic shapes of PB and BM cellsCRITERIA OF AAEXCLUSIVE EVIDENCES:No evidence