Pathogenesis of Beta Thalassemia and associated Hemoglobinopathies Normal ad emeabin Hs + "SeHbA [acbB = 2a+2B chaos) DHA, 956 2% HBF aay ea hemeloin) Ok aap tupercrps show (etre made; + means some Fetus makes HBF (ay) which has very high (,afity HOF takes, from mom's Hb In placenta; no chains required “ narestcty monet stim afte ‘A ma, nfant makes more HDA than HOF: aaa eeeee recmowes coreoing esuanng oy postive seecton presse > ‘metal therepy sruatons pion preaters Ed | ‘Anemia of Chronic Disease: Pathophysiology and Clinical Manifestations ‘ne theory 0 expla why anemia of The ammaow eke respons acta often equie xan as natiet or Sarva Thabo byt ck up ron ‘Chronic sat (fection, autoimmune conditions, "stone eracnce malignancies, metabolic diseases, hear fle) ¥ Release of inflammatory tines ae thaw Sores tne of ote neon ay 4 ryteopoetn ra halp oon baer erowen {EPO} production and WL stimuletes nero reduce hsacdin —jeepenseta. release rom kdneys + erythropoietin (EPO) Long-term nowere resus in ane vain is een te Gi meeps “ree oon om REET, ewopoae eres enor th pu (rescloedotea sam) ry x “heme snthesis 4b entropies (RBC Synthese the Bone marrow ¥ ‘tl suficint evel of ron aval toll whatever RECs there ae with roma amcunt of hemogltin + ‘Nomosycanemiasickle Cell Disease: Signs and Symptoms “SEES meets ~ 20% vane 23o* rc ships nse dotothe ieee) Df sesouen Dolmerztonsf mitted — npr! 20.28% Aesaner els Tiecharemogabes —” Beediment GOO S| “oesie re eet since \msoacton tree hamoe EET (idl Crip Oona acophagesnteapen —v, Menno velaaesl a= imatenst Noging > “lng puna Pogson wee noc) ~~ sfnttowtoramand "Mood pene wet ae + pene ia fhidewanantesine —‘ RBCBEIdown ‘tmarowyuoplto ! ‘ ‘raat te pigmented fecinennet cto an nape 4 ‘taore | fmedbyssces |“ teculeg eaegate Gmina) frees acts + paises eer | (oteund anes) conten) gah atin obloodsmanr” Meanie Sepe ‘een pathopnsiooay Mechanism | Sn/Sympton/iab fing conpkators | PillshedSeptenberis,2013onwrumeaeaneuseon EOS Glucose-6-phosphate dehydrogenase (G6PD) deficiency: webct Pathogenesis and clinical findings ioe ETRE a a ae hhexose monophosphate shunt of yeolsis ae eo MD atime of ‘Surmasnneadamnte storage ceded GSPD ere 28) mttoncetsin atc _lutatione (63H) to protect red blod cls 1 65D production in REC {hac om onthe damage + ot ge ot 1 eitaton (6 regeneration sears “Linfecion _, eenaepataas ely of RCo rest onion (eos) "eee ; tac hieion net expire Po 2 onan ds 1 detent no reas noe (amma rs tinding” 7 “Sungottobn aan “namie, ecco heme sein) f + Fava beans generate 3.Favabeans —> "ondantewhan Precipitated H(Heinz body) binds ‘metabolzed toand deforms RBC membrane RAC membrane deformities telager hemolysis inthe blood ‘est intravascular) andin the ‘splen estravasulr) + Hemotsin GEPD deen ocuts rea + ae ‘No findings of chronic hemolysis ‘no [suchas splnometal ond pamented ‘eset welizones) i ( o8e- Ee o. Precipitated Hb forms meme ‘oun ineston Bader in DC Heinz bodies show vo on ood ung supra tan Macrophages the spleen cs wth remove az bodyportonfthe —> sence gerton remove) BC memtrane ‘peripheral aod fen ErSRES] =e -Ee aa aE ‘result of 7 Hb breakdown in the spleen Jaundice = Ee =o Hoptolbin binds to ree Ho. Hapapoin is ‘pstedarHapteslobn Hbcomplctes sre —» L Haptoglobin removed by the etiloendlheat ten ‘Normocytic anemia legend: Pathophysiology Mechanism Sgn/Symptom/Lab Fading Completions | P ed November 20° 2042 on www thecakaryguide com