地中海贫血和血红蛋白胰岛素血红蛋白病 Thalassemia and Hemoglobinopathy典型实例.pptVIP

Primary Laboratory InvestigationThalassemia Severe cases present with Microcytosis Hypochromia Poikilocytosis RBC counts higher than expected for the level of anemia Primary Laboratory InvestigationThalassemia Findings in severe cases can mimic those seen in other microcytic/hypochromic anemias Results of the reticulocyte count are variable NRBCs may be present (contrast with iron deficiency anemia) Primary Laboratory InvestigationHemoglobinopathy Variety of hemogram findings depending on Type Severity of the specific disorder Only sickle hemoglobinopathies and Hb C will be described here Primary Laboratory InvestigationHeterozygous Other Disorders AS S-Thal Other hemoglobinopathies, e.g., SC Hb C  Morphologic Findings Hb SS vs. Hb SC vs. Hb CC = + Hb S Hb C Hb SC Morphologic Findings Hb SS vs. Hb SC vs. Hb CC = + Hb S Hb C Hb SC + = Where Do Sickle Cells Come From? Sheared in microcirculation Irreversible Sickle Cell Sickle Cells Secondary Laboratory Investigation Hemoglobin electrophoresis Major test for identifying thalassemia and hemoglobinopathy Types Cellulose acetate: Alkaline pH Citrate agar: Acid ph Secondary Laboratory Investigation Patterns of mobility (see Fig. 14-4, Clinical Hematology, p. 202) Limitations Sources of error Secondary Laboratory InvestigationCellulose Acetate Hb Electrophoresis - A2/C S F A + Normal Secondary Laboratory InvestigationCellulose Acetate Hb Electrophoresis - A2/C S F A + Normal Hb SS Secondary Laboratory InvestigationCellulose Acetate Hb Electrophoresis - A2/C S F A+ Normal Hb SS Hb AS Secondary Laboratory InvestigationCellulose Acetate Hb Electrophoresis - A2/C S F A+ Normal Hb SS Hb AS Hb SC Secondary Laboratory InvestigationCellulose Acetate Hb Electrophoresis - A2/C S F A+ Normal Hb SS Hb AS Hb SC Hb CC THALASSEMIAS AND HEMOGLOBINOPATHIES CLS 843 ADVANCED CLINICAL HEMATOLOGY Raymon