Aplastic anemiaCONTENTSINTRODUCTIONETIOLOGY /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 pancytopenia• Incidence: 7.4 per million population East > West Biphasic peak at 20-25 and >60 yCauses: Inherited (20%)• Fanconi anemia• Dyskeratosis congenita• Cartilage hair hypoplasia• Pearson syndrome• Amegakaryocytic thrombocytopenia• Shwachman-Diamond syndrome• Dubowitz syndrome• Diamond-Blackfan syndrome• Familial aplastic anemiaCauses: Acquired (80%) • Infections: hepatitis, EBV, HIV, parvovirus• Radiation and chemicals such as benzene• Drugs: chloramphenicol, phenylbutazone ,gold• PNH (paroxysmal nocturnal hemoglobinuria)• Transfusional graft versus host disease• Liver transplantation• PregnancyPathogenesis• Depletion of hemopoietic stem cell• Suppression of proliferation and maturation Mechanism -Immune related -Cytotoxic T-cell, INF (Interferon ) γ, TNF(tumor necrosis factor )α↑Presentation• Anemia: pallor, headache, palpitations, dyspnea, heart failure• Thrombocytopenia: mucosal and gingival bleeding or petechia• Neutropenia: infections, mouth and pharyngeal ulcerationsTests• PB, BM( multiple) smear, biopsy, cytochemistry• Chromosome• T cell number and function• Others 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 AA• Criteria direct evidences exclusive evidences• Subtypes SAA (severe AA) VSAA (very severe AA) NSAA (not severe AA)CRITERIA OF AA• DIRECT EVIDENCES:– Pancytopen...
