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What is Eosin-5-Maleamide flow cytometry is used for diagnosis of: and explain in detail?
Ans: (b) Hereditary spherocytosisRef: Wintrobe's Hematology, compiledHereditary SpherocytosisAD inheritanceMost common mutation - Ankyrin*Most severe mutation Spectrin*The spherocytes of HS have 2 defects:Reduced deformabilityReduced membrane plasticityPeripheral smearSpherocytesHowell jolly bodies - post splenectomyHemolysis - ExtravascularDiagnosisPeripheral smearOsmotic fragility test - ScreeningGold standard - Flow cytometry - EMA (Eosin-5-Maleamide) Flowcytometry EMA FlowcytometryA flowcytometric test based on eosin-5-maleamide (EMA) which binds to band 3 of red cell membrane is a sensitive and specific test for diagnosis of HS.The mean fluorescence intensity of EMA tagged red cells is lower in HS (MCF<0.80).EMA fluorescence is normal or increased in AIHA (DD for spherocytosis).