Head of Flow cytometry unit Assistant professor amp Consultant Hematologists Hemoglobin α β β α Fe Fe Fe Fe Globin chain Haem Prophyrin ring Iron atom ID: 1033133
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1. ANEMIA Dr. Mansour AljabryHead of Flow cytometry unitAssistant professor & Consultant Hematologists
2. Hemoglobin??
3. αββαFe⁺⁺Fe⁺⁺Fe⁺⁺Fe⁺⁺Globin chainHaemProphyrin ring Iron atomO2O2O2O2Hemoglobin structureDr. Aljabry
4. Hemoglobin Hemoglobin is the protein molecule in RBC that carries O2 from the lungs to the body's tissues and returns carbon CO2 from the tissues back to the lungs.Hemoglobin maintains the shape of RBC also.
5. Hematopoietic stem cell:1- Self renewal2- Cell differentiation HematopoiesisMyeloid SCErythroid Precursors Transcriptional FactorErythropoietin GATA1
6. 66Late NormoblastReticulocyteErythrocyteBasophilicNormoblastErythroblastIntermediateNormoblast+++++++++-Synthesis of HemoglobinErythropoiesis The “Bone Marrow” is the major site with the need of:Folic acid – Iron “Ferrous” – Vit B12 – Erythropoietin -Amino acidsminerals - other regulatory factors
7. FemaleMaleIndices11.5-15.513.5-17.5Hemoglobin(g/dL)36-4840-52Hematocrit (PCV) (%)3.9-5.64.5-6.5Red Cell Count (×10¹²)80-95Mean Cell Volume (MCV) (fL)30-35Mean Cell Hemoglobin (MCH) (pg)HbMacrocyticNormocyticMicrocyticMCVNormochromicHypochromicMCHNormal RangesHCT
8. An (without) -aemia (blood) Reduction of Hb concentration below the normal range for the age and genderLeading to decreased O2 carrying capacity of blood and thus O2 availability to tissues (hypoxia)ANEMIA
9. Clinical FeaturesPresence or absence of clinical feature depends on:1-Speed of onset : Rapidly progressive anemia causes more symptoms than slow onset anemia due to lack of compensatory mechanisms: (cardiovascular system, BM &O2 dissociation curve2-Severity: Mild anemia :no symptoms usually Symptoms appear if Hb less than 9g/dL3- Age: Elderly tolerate anemia less than young patients
10. WeaknessHeadache Pallor LethargyDizzinessPalpitation (tachycardia)AnginaCardiac failure Related to anemiaRelated to compensatory mechanismClinical Features1-General features of anemiaSpecific signs are associated with particular types of anemia :Spoon nail with iron deficiency, Leg ulcers with sickle cell anemia Jaundice with hemolytic anemia bone deformities in thalassemia major2-Specific features
11. Classification of Anemia
12. Hypochromic microcytic anemia HemoglobinProphyrin IronGlobin chain ThalassemiaIron def. anemiaSidroblastic anemiaDNA DNA synthesisMegaloblastic anemia: -B12 def. -Folate def. MDSMacrocytic anemia RBC count HemolysisRBC productionAcute bleedingAutoimmuneEnzymopathyMembranopathyMechanicalSickle cell anemia BM failure:-Chemotherapy-Aplastic anemia-MalignancyAnemia of chronic diseaseBlood lossNormocytic normochromic anemia
13. Iron is among the abundant minerals on earth (6%).Iron deficiency is the most common disorder( 24%).Limited absorption ability : 1-Only 5-10% of taken iron will be absorbed 2- Inorganic iron can not be absorbed easily.Excess loss due to hemorrhage Iron Deficiency Anemia!
14. 28/01/143714Daily absorption ≈1 mg Circulating hemoglobin(2.5g)Bone marrow erythroblast (150mg)Daily loss ≈1 mg Liver and muscle myoglobin (650mg)Urine faecesSkinnailhairTransferrin (4mg)menstrual loss(hemorrhage)Macrophage (1g)Storage forms:FerritinHaemosiderin
15. HepcidinBM macrophage- veIL6Tfr2+veHypoxiaIron for erythropoeisis
16. Factor reducing absorption Factors favoring absorptionInorganic iron Haem ironFerric iron Fe+++Ferrous Iron (Fe++)AlkalinesAcid Iron overload Iron defTeaPregnancyIncreased hepcidinHemochromatosisPrecipitating agent(phenol)Solubilizing agent (Sugar)Iron Absorption
17. 1-Body Iron status: Increased demands(iron def.,pregnancy..)Low iron stores high absorption Iron overload Full iron storesLow absorption2- Content and form of dietary iron More Iron Heam Iron More absorption 3- Balance between dietary enhancers&Inhibitory factors:Meat (haem iron) Fruit (Vitamin C)Sugar (Solubilizing agent )AcidsDairy foods (calcium) High fiber foods (phytate) Coffee &tea (polyphenoles)Anti -Acids EnhancersInhibitorsIron AbsorptionFerrous Iron
18. 1-Chronic blood loss:GIT Bleeding: peptic ulcer, esophageal varices , hookworm cancerUterine bleedingHematuria2- Increased demands:ImmaturityGrowthPregnancyEPO therapy3-Malabsorption: Enteropathy Gastrectomy4-Poor diet: Rare as the only cause (rule out other causes)Causes of IDA
19. 4Iron def. anemia 3Latent2Pre-latent1NormalLowLowLowNormal StoresLowLowNormal Normal MCV/MCHLow Normal NormalNormalHemoglobinDevelopment of IDASigns of anemia
20. 20Beside symptoms and signs of anaemia +/- bleeding patients present with:(a): Koilonychia (spoon-shaped nails)(b): Angular stomatitis and/or glossitis (c): Dysphagia due to pharyngeal web (Plummer-Vinson syndrome)Signs and symptoms of IDAacb
21. InvestigationMicrocytic hypochromic anemia with:Anisocytosis( variation in size)Pokiliocytosis (variation in shape)normal
22. Iron StudiesTIBC*Serum IronSerum ferritinTransferrin saturation Low serum ironLow serum ferritinLow transferrin saturation high TIBCNormalIDATIBC : total iron binding capacity of transferrin
23. Iron Studies Low TIBC*High Serum IronHigh Serum ferritinHigh Transferrin saturation Low serum ironLow serum ferritinLow transferrin saturation high TIBCThalassemiaIDANormal
24. BM Iron stain (Perl’s stain): The gold standard but invasive procedure NormalIDA: reduced or absent iron stores(hemosiderin)Investigation
25. Treatment of IDATreat the underlying causeIron replacement therapy: Oral :( Ferrous Sulphate OD for 6 months)Intravenous:( Ferric sucrose OD for 6 months)Hb should rise 2g/dL every 3 weeks
26. PREVENTION OF IDADietary modification Meat is better source than vegetables. Food fortification (with ferrous sulphate)GIT disturbances ,staining of teeth & metallic taste.Iron supplementation: For high risk groups.
27. Anemia of chronic diseaseNormochromic normocytic (usually) anemia caused by decreased release of iron from iron stores due to raised serum Hepcidin .Associated with - Chronic infection including HIV, malaria - Chronic inflammations -Tissue necrosis -Malignancy
28. HepcidinBM macrophageIL-6IL-1TNF no Iron for erythropoeisis+veTuberculosisSLECarcinoma Lymphoma- ve
29. 29Management:Treat the underlying causeIron replacement +/- EPONormocytic normochromic or mildly microcytic anaemiaLow serum iron and TIBCNormal or high serum ferritin ( acute phase reactant)High haemosiderin in macrophages but low in normoblasts Work-up and treatment