2 E. DONNALL THOMAS, M.D. Nobel Prize in Physiology or Medicine, 1990 for his pioneering work on bone marrow transplantation
3 造血幹細胞移植的歷史1957: Thomas ED et.al.: st attempt of BMT in human beings (failure; HLA was identified in 1960+)1959: Thomas ED et.al.: nd attempt of BMT in identical twin(hematological recovery occurred in 2 weeks, butleukemia recurred months later)
4 Allogeneic HSCT, for What? To replace incorrigible hematopoietic defects:Quantitative defect: 再生不良性貧血/Fanconi’s anemiaQualitative defect:惡性疾病: 各種急性或慢性,骨髓性或淋巴球性白血病,淋巴癌,多發性骨髓瘤非惡性疾病:骨髓造血幹細胞缺陷疾病:重度免疫不全症候造血細胞基因缺損: 嚴重型地中海型貧血酵素相關基因缺陷: 大理石症,高雪氏症,黏寶寶
22 Tests for chimerism Erythrocyte Ag (ABO, Rh, MN…) Cytogenetics of metaphase for sex chromosomeFISHSTR(microsatellite)/VNTR(minisatellite) of nuclear cells—even lineage-specific STR analysis (after FACS with sorting)
32 Changing Concepts: GVHD Syndrome After AlloHCT Acute GVHD: rash, GI, liver Chronic GVHD: skin, eyes, mouth, GIliver, musculoskeletal, lungs, GUAlloreactivityAutoimmunityImmunodeficiency- Classic acute Late acute - Classic chronic- Chronic overlapAll Images Are Copyright ProtectedDay y 2 y y yActivity Damage(inflammation) i n j u r y r e p a i r (fibrosis)
33 Acute GVHD Engraftment to D+100 Alloreactive T-cells against HLA-bearing host tissueGeneral performance decreasedSkin rashOral mucositisGastritisColitisHepatobiliary: hepatitis, jaundiceCytopenia, thrombocytopeniaImpaired immunity
39 Infections Disability Quality of life Endocrine Metabolism Nutrition Bronchiolitis obliteransOcular siccaLoss of bile ductsOral ulcersNail dystrophyFasciitisInfectionsDisabilityQuality of lifeEndocrineMetabolismNutritionPainSkin sclerosisSkin ulcersSpectrum ofmanifestationsin chronic GVHDAll Images Are Copyright ProtectedDeep sclerosis
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