Hematology PPT- anemia, thalasemia, sickle cell anemia
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This document summarizes several hematological disorders including anemia, sickle cell anemia, and thalassemia. It discusses the origins and functions of blood, classifications of anemia, causes and symptoms of iron deficiency anemia, and treatments including oral iron therapy and blood transfusions. For sickle cell anemia, it covers alleles, pathophysiology, clinical features like vaso-occlusive crises, and treatments including pain management, oxygenation, and antibiotics. For thalassemia, it discusses decreased synthesis of hemoglobin chains, classifications, clinical features such as prominent facial bones and enlarged organs, diagnosis, and treatments including blood transfusions and iron chelation therapy.
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Hematology PPT- anemia, thalasemia, sickle cell anemia
- 2. Origin Of fOrmed elements
- 3. fUnCtiOns Of BlOOd • TRANSPORT OF O2 AND CO2 • IMMUNE FUNCTION • COAGULATION • MAINTAINS BLOOD PH
- 4. ANEMIA
- 5. Classification According To WHO Grading
- 8. Common Anemia In Children • Nutritional Anemia-IDA, Megaloblastic Anemia • SickleCell Anemia • Thalassemia
- 9. IRON DEFICIENCY ANEMIA • It Is A Type Of Microcytic Hypo Chromic Anaemia Results From Inadequate Supply Of Dietary Iron In The Body.
- 10. Causes
- 11. PATHOPHYSIOLOGY Supply of inadequate iron Depletion of iron stores Depletion of serum transferrin concentration Subnormal Hb concentration Subnormal oxygen carrying capacity
- 12. CLINICAL MANIFESTATIONS • Pallor, Anorexia, Irritability • Palpitation, shortness of breath, fatigue • Atrophy of tongue • Koilonychias in older children • Organomegaly • Lack of attention span • Pica • Growth retardation • Impaired mental and physical functions
- 13. • History collection • Physical examination • Laboratory investigations • Bone marrow studies
- 14. Diagnosis Laboratory investigations • Peripheral blood smear shows microcytic hypo chromic RBC • Reduced reticulocyte count • Hb % is usually low • Low serum iron=<75 mg/dl • Increased TIBC=>470 mg/dl • Decreased serum ferritin level =<15mg/ml
- 15. MANAGEMENT
- 16. Oral iron therapy • Recommended Dosage • Infant and children:3 mg/kg/day • 20mg elemental iron +100 mcg folic acid • Adolescent :100 mg elemental iron +500 µg of folic acid +25-50 mg vit.c Mild Anemia: Once Daily Moderately And Severe Anemia: Twice Daily Mild Anemia: Once Daily Moderately And Severe Anemia: Twice Daily Result Peak increase of reticulocyte count in 5th -10th day of administration Result Peak increase of reticulocyte count in 5th -10th day of administration
- 17. Oral iron therapy PREPARATION ELEMENTALIRON/TAB IRON ABSORPTION /TAB Ferrous sulfate 60 mg 20 mg Ferrous fumerate 66 mg 33 mg Ferrous gluconate 36 mg 12 mg
- 18. • Iron dextran complex:50 mg/ml in 2ml saline • Dose calculation: • Route=intramuscular---Z track method intravenous-----infusion methods Iron [mg]= Wt. in kg x Hb deficit [gm/dl] x4
- 19. Transfusion therapy • Given when Hb is <3-4 gm/dl • Packed red cell 2-3 ml/kg Note: Infuse at slow rate to prevent cardiac overload, a dose of frusemide 1-2mg/kg IV is given during transfusion
- 20. Diet therapy • Good sources of iron: • legumes • nuts, • Green leafy veg. • dates, • yolk of egg,fish, meat, liver, • ripe banana, mango • Cooking in iron pots • Poor source of iron: • Milk • Administer iron with fruit juices • iron with milk or antacids
- 21. Health education • Diet counseling for parents • Iron fortified formulas • Limitation of milk • Avoid beverages like tea • Colour changes of stool • Staining of teeth • Deworming
- 23. Definition • Sickle Cell Anemia Is An Autosomal Recessive Order In Which Normal Adult Hb A Is Partly Or Completely Replaced By Abnormal Sickle Hb[Hb S]
- 24. Alleles • Homozygous-same identical alleles • Heterozygous- two different alleles AA, rr Ar
- 26. PATHOPHYSIOLOGY
- 27. Change in molecular stability & solubility Tactoids Insoluble fibre like structures Deformation of RBC VASOOCCLUSION Adjacent Tissue Ischemia
- 31. VASO OCCLUSIVE CRISIS • ABDOMINAL PAIN • OBSTRUCTIVE JAUNDICE • HEMATURIA • PRIAPISM
- 34. APLASTIC CRISIS • DIMINISHED PRODUCTION OF RBC • ACCELERATED RATE OF RBC DESTRUCTION • DUE TO BLOCKAGE OF MAJOR VESSELS HYPERHEMOLYTIC CRISIS CVA
- 35. Normal chest ray Acute chest syndrome
- 37. Diagnosis • New born screening • Chronic villus biopsy • Hb electrophoresis • Sickle turbidity test • Iso electric focusing • High performance liquid chromatography • Globin DNA analysis
- 40. Treatment Medical management • Supportive therapies • Symptomatic management Surgical management • Spleenectomy • Surgical exploration of abdomen
- 41. Medical management Oxygenation Rest Hydration Pain management Blood transfusion Antibiotics Gene therapy Folic acid supplementation Electrolyte replacement
- 42. Alleviating Pain Warmth: increases blood flow Massaging and rubbing Hot fomentation Bandaging Rest Analgesics Cognitive Behavioral Therapy Relaxation therapy
- 43. THALASSEMIA Thalassemia is an autosomal recessive, characterized by decreased synthesis of one of the polypeptide chains [ or ]α β that form normal adult haemoglobin molecule
- 44. Incidence Mediterranean ancestry, blacks and Southern china, South East Asia. In South East Asia it is 40 million In India its 50 %
- 45. Main Types
- 46. Classification • β thalassemia • Silent carrier • Thalassemia major • Thalassemia intermedia • Thalassemia minor • α thalassemia • Silent Carrier • α Thalassemia trait • Hb H Disease • Hydrops fetalis
- 47. Causes
- 51. Contd.. • PROMINENT FRONTAL AND PARIETAL BOSSING • MALOCCLUSION OF TEETH
- 52. Contd…. 1. Pale skin or jaundice 2. Protruding abdomen 3. Enlarged spleen and liver 4. Dark urine 5. Fatigue
- 53. diagnosis • Prenatal testing • Peripheral blood film examination • Complete blood count (CBC)
- 54. • Hemoglobin studies. • Family genetic studies • Radiological findings-hair end appearance • NESTROFT TEST
- 56. Blood transfusion • Type :saline washed packed red cells • Rate :10-15 ml/kg
- 57. Methods of transfusion Method Hb level Maintenance level Palliative <7gm% <8.5 gm% Hyper transfusion >10 gm% >12 gm% Super transfusion >12mg% >14gm% Moderate transfusion 9-10gm% >12gm% Post transfusion Hb level should not be >16gm/dl
- 58. Iron Chelation therapy Drugs used are: • Desferrioxamine:30-40 mg/kg/day • Deferiprone:75-100 mg/kg/day • Desferasirox :20 -40mg/kg/day When to start? After 10-20 blood transfusion
- 59. Thank you….