HEMATOLOGICAL DISORDERS
Origin Of fOrmed elements
fUnCtiOns Of BlOOd• TRANSPORT OF O2 AND CO2• IMMUNE FUNCTION• COAGULATION• MAINTAINS BLOOD PH
ANEMIA
Classification According To WHO Grading
CLASSIFICATION
Common Anemia In Children• Nutritional Anemia-IDA, MegaloblasticAnemia• SickleCell Anemia• Thalassemia
IRON DEFICIENCY ANEMIA• It Is A Type Of Microcytic Hypo ChromicAnaemia Results From Inadequate SupplyOf Dietary Iron In Th...
Causes
PATHOPHYSIOLOGYSupply of inadequate ironDepletion of iron storesDepletion of serum transferrin concentrationSubnormal Hb c...
CLINICALMANIFESTATIONS• Pallor, Anorexia, Irritability• Palpitation, shortness of breath, fatigue• Atrophy of tongue• Koil...
• History collection• Physical examination• Laboratory investigations• Bone marrow studies
DiagnosisLaboratory investigations• Peripheral blood smear shows microcytic hypo chromicRBC• Reduced reticulocyte count• H...
MANAGEMENT
Oral iron therapy• Recommended Dosage• Infant and children:3 mg/kg/day• 20mg elemental iron +100 mcg folic acid• Adolescen...
Oral iron therapyPREPARATION ELEMENTALIRON/TAB IRON ABSORPTION/TABFerrous sulfate 60 mg 20 mgFerrous fumerate 66 mg 33 mgF...
• Iron dextran complex:50 mg/ml in 2ml saline• Dose calculation:• Route=intramuscular---Z track methodintravenous-----infu...
Transfusiontherapy• Given when Hb is <3-4 gm/dl• Packed red cell 2-3 ml/kgNote:Infuse at slow rate to prevent cardiacoverl...
Diet therapy• Good sources of iron:• legumes• nuts,• Green leafy veg.• dates,• yolk of egg,fish, meat, liver,• ripe banana...
Healtheducation• Diet counseling for parents• Iron fortified formulas• Limitation of milk• Avoid beverages like tea• Colou...
Definition• Sickle Cell Anemia Is An AutosomalRecessive Order In Which NormalAdult Hb A Is Partly Or CompletelyReplaced By...
Alleles• Homozygous-same identical alleles• Heterozygous- two different allelesAA, rrAr
PATHOPHYSIOLOGY
Change in molecularstability & solubilityTactoidsInsoluble fibre likestructuresDeformation of RBCVASOOCCLUSIONAdjacent Tis...
Clinical features
VASO OCCLUSIVE CRISIS• ABDOMINAL PAIN• OBSTRUCTIVE JAUNDICE• HEMATURIA• PRIAPISM
Clinical features
ACUTE SPENIC SEQUESTRATION
APLASTIC CRISIS• DIMINISHED PRODUCTION OF RBC• ACCELERATED RATE OF RBCDESTRUCTION• DUE TO BLOCKAGE OF MAJOR VESSELSHYPERHE...
Normal chest ray Acute chest syndrome
Clinical features
Diagnosis• New born screening• Chronic villus biopsy• Hb electrophoresis• Sickle turbidity test• Iso electric focusing• Hi...
TreatmentMedical management• Supportive therapies• Symptomatic managementSurgical management• Spleenectomy• Surgical explo...
Medical managementOxygenationRestHydrationPain managementBlood transfusionAntibioticsGene therapyFolic acid supplementatio...
Alleviating Pain Warmth: increases blood flow Massaging and rubbing Hot fomentation Bandaging Rest Analgesics Cogni...
THALASSEMIAThalassemia is an autosomal recessive,characterized by decreased synthesis ofone of the polypeptide chains [ or...
IncidenceMediterranean ancestry, blacks andSouthern china, South East Asia.In South East Asia it is 40 millionIn India its...
Main Types
Classification• β thalassemia• Silent carrier• Thalassemia major• Thalassemia intermedia• Thalassemia minor• α thalassemia...
Causes
Pathophysiology OfThalassemia
Clinical features
Contd..• PROMINENT FRONTALAND PARIETALBOSSING• MALOCCLUSION OFTEETH
Contd….1. Pale skin or jaundice2. Protruding abdomen3. Enlarged spleen and liver4. Dark urine5. Fatigue
diagnosis• Prenatal testing• Peripheral blood filmexamination• Complete blood count(CBC)
• Hemoglobin studies.• Family genetic studies• Radiological findings-hair end appearance• NESTROFT TEST
Blood transfusion• Type :saline washed packed redcells• Rate :10-15 ml/kg
Methods of transfusionMethod Hb level Maintenance levelPalliative <7gm% <8.5 gm%Hyper transfusion >10 gm% >12 gm%Super tra...
Iron Chelation therapyDrugs used are:• Desferrioxamine:30-40 mg/kg/day• Deferiprone:75-100 mg/kg/day• Desferasirox :20 -40...
Thank you….
Hematology PPT- anemia, thalasemia, sickle cell anemia
Hematology PPT- anemia, thalasemia, sickle cell anemia
Hematology PPT- anemia, thalasemia, sickle cell anemia
Hematology PPT- anemia, thalasemia, sickle cell anemia
Hematology PPT- anemia, thalasemia, sickle cell anemia
Hematology PPT- anemia, thalasemia, sickle cell anemia
Hematology PPT- anemia, thalasemia, sickle cell anemia
Hematology PPT- anemia, thalasemia, sickle cell anemia
Hematology PPT- anemia, thalasemia, sickle cell anemia
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Hematology PPT- anemia, thalasemia, sickle cell anemia

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Hematology PPT- anemia, thalasemia, sickle cell anemia

  1. 1. HEMATOLOGICAL DISORDERS
  2. 2. Origin Of fOrmed elements
  3. 3. fUnCtiOns Of BlOOd• TRANSPORT OF O2 AND CO2• IMMUNE FUNCTION• COAGULATION• MAINTAINS BLOOD PH
  4. 4. ANEMIA
  5. 5. Classification According To WHO Grading
  6. 6. CLASSIFICATION
  7. 7. Common Anemia In Children• Nutritional Anemia-IDA, MegaloblasticAnemia• SickleCell Anemia• Thalassemia
  8. 8. IRON DEFICIENCY ANEMIA• It Is A Type Of Microcytic Hypo ChromicAnaemia Results From Inadequate SupplyOf Dietary Iron In The Body.
  9. 9. Causes
  10. 10. PATHOPHYSIOLOGYSupply of inadequate ironDepletion of iron storesDepletion of serum transferrin concentrationSubnormal Hb concentrationSubnormal oxygen carrying capacity
  11. 11. CLINICALMANIFESTATIONS• 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
  12. 12. • History collection• Physical examination• Laboratory investigations• Bone marrow studies
  13. 13. DiagnosisLaboratory investigations• Peripheral blood smear shows microcytic hypo chromicRBC• Reduced reticulocyte count• Hb % is usually low• Low serum iron=<75 mg/dl• Increased TIBC=>470 mg/dl• Decreased serum ferritin level =<15mg/ml
  14. 14. MANAGEMENT
  15. 15. 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 mgvit.cMild Anemia: Once DailyModerately And Severe Anemia: TwiceDailyMild Anemia: Once DailyModerately And Severe Anemia: TwiceDailyResultPeak increase of reticulocyte count in 5th-10thday of administrationResultPeak increase of reticulocyte count in 5th-10thday of administration
  16. 16. Oral iron therapyPREPARATION ELEMENTALIRON/TAB IRON ABSORPTION/TABFerrous sulfate 60 mg 20 mgFerrous fumerate 66 mg 33 mgFerrous gluconate 36 mg 12 mg
  17. 17. • Iron dextran complex:50 mg/ml in 2ml saline• Dose calculation:• Route=intramuscular---Z track methodintravenous-----infusion methodsIron [mg]= Wt. in kg x Hb deficit [gm/dl] x4
  18. 18. Transfusiontherapy• Given when Hb is <3-4 gm/dl• Packed red cell 2-3 ml/kgNote:Infuse at slow rate to prevent cardiacoverload, a dose of frusemide 1-2mg/kg IVis given during transfusion
  19. 19. 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 withfruit juices• iron with milk orantacids
  20. 20. Healtheducation• Diet counseling for parents• Iron fortified formulas• Limitation of milk• Avoid beverages like tea• Colour changes of stool• Staining of teeth• Deworming
  21. 21. Definition• Sickle Cell Anemia Is An AutosomalRecessive Order In Which NormalAdult Hb A Is Partly Or CompletelyReplaced By Abnormal Sickle Hb[Hb S]
  22. 22. Alleles• Homozygous-same identical alleles• Heterozygous- two different allelesAA, rrAr
  23. 23. PATHOPHYSIOLOGY
  24. 24. Change in molecularstability & solubilityTactoidsInsoluble fibre likestructuresDeformation of RBCVASOOCCLUSIONAdjacent Tissue Ischemia
  25. 25. Clinical features
  26. 26. VASO OCCLUSIVE CRISIS• ABDOMINAL PAIN• OBSTRUCTIVE JAUNDICE• HEMATURIA• PRIAPISM
  27. 27. Clinical features
  28. 28. ACUTE SPENIC SEQUESTRATION
  29. 29. APLASTIC CRISIS• DIMINISHED PRODUCTION OF RBC• ACCELERATED RATE OF RBCDESTRUCTION• DUE TO BLOCKAGE OF MAJOR VESSELSHYPERHEMOLYTIC CRISISCVA
  30. 30. Normal chest ray Acute chest syndrome
  31. 31. Clinical features
  32. 32. Diagnosis• New born screening• Chronic villus biopsy• Hb electrophoresis• Sickle turbidity test• Iso electric focusing• High performance liquid chromatography• Globin DNA analysis
  33. 33. TreatmentMedical management• Supportive therapies• Symptomatic managementSurgical management• Spleenectomy• Surgical exploration of abdomen
  34. 34. Medical managementOxygenationRestHydrationPain managementBlood transfusionAntibioticsGene therapyFolic acid supplementationElectrolyte replacement
  35. 35. Alleviating Pain Warmth: increases blood flow Massaging and rubbing Hot fomentation Bandaging Rest Analgesics Cognitive Behavioral Therapy Relaxation therapy
  36. 36. THALASSEMIAThalassemia is an autosomal recessive,characterized by decreased synthesis ofone of the polypeptide chains [ or ]α βthat form normal adult haemoglobinmolecule
  37. 37. IncidenceMediterranean ancestry, blacks andSouthern china, South East Asia.In South East Asia it is 40 millionIn India its 50 %
  38. 38. Main Types
  39. 39. Classification• β thalassemia• Silent carrier• Thalassemia major• Thalassemia intermedia• Thalassemia minor• α thalassemia• Silent Carrier• α Thalassemia trait• Hb H Disease• Hydrops fetalis
  40. 40. Causes
  41. 41. Pathophysiology OfThalassemia
  42. 42. Clinical features
  43. 43. Contd..• PROMINENT FRONTALAND PARIETALBOSSING• MALOCCLUSION OFTEETH
  44. 44. Contd….1. Pale skin or jaundice2. Protruding abdomen3. Enlarged spleen and liver4. Dark urine5. Fatigue
  45. 45. diagnosis• Prenatal testing• Peripheral blood filmexamination• Complete blood count(CBC)
  46. 46. • Hemoglobin studies.• Family genetic studies• Radiological findings-hair end appearance• NESTROFT TEST
  47. 47. Blood transfusion• Type :saline washed packed redcells• Rate :10-15 ml/kg
  48. 48. Methods of transfusionMethod Hb level Maintenance levelPalliative <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
  49. 49. Iron Chelation therapyDrugs used are:• Desferrioxamine:30-40 mg/kg/day• Deferiprone:75-100 mg/kg/day• Desferasirox :20 -40mg/kg/dayWhen to start?After 10-20 blood transfusion
  50. 50. Thank you….

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