• Like
  • Save
Hematology PPT- anemia, thalasemia, sickle cell anemia
Upcoming SlideShare
Loading in...5
×

Hematology PPT- anemia, thalasemia, sickle cell anemia

  • 9,878 views
Uploaded on

Under the guidance of our HOD Asso:Professor Mrs.Deepa Danieal

Under the guidance of our HOD Asso:Professor Mrs.Deepa Danieal

  • Full Name Full Name Comment goes here.
    Are you sure you want to
    Your message goes here
  • i need to download this.hlp me
    Are you sure you want to
    Your message goes here
No Downloads

Views

Total Views
9,878
On Slideshare
0
From Embeds
0
Number of Embeds
3

Actions

Shares
Downloads
0
Comments
1
Likes
34

Embeds 0

No embeds

Report content

Flagged as inappropriate Flag as inappropriate
Flag as inappropriate

Select your reason for flagging this presentation as inappropriate.

Cancel
    No notes for slide

Transcript

  • 1. HEMATOLOGICAL DISORDERS
  • 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
  • 6. CLASSIFICATION
  • 7. Common Anemia In Children• Nutritional Anemia-IDA, MegaloblasticAnemia• SickleCell Anemia• Thalassemia
  • 8. IRON DEFICIENCY ANEMIA• It Is A Type Of Microcytic Hypo ChromicAnaemia Results From Inadequate SupplyOf Dietary Iron In The Body.
  • 9. Causes
  • 10. PATHOPHYSIOLOGYSupply of inadequate ironDepletion of iron storesDepletion of serum transferrin concentrationSubnormal Hb concentrationSubnormal oxygen carrying capacity
  • 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. • History collection• Physical examination• Laboratory investigations• Bone marrow studies
  • 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. MANAGEMENT
  • 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. Oral iron therapyPREPARATION ELEMENTALIRON/TAB IRON ABSORPTION/TABFerrous sulfate 60 mg 20 mgFerrous fumerate 66 mg 33 mgFerrous gluconate 36 mg 12 mg
  • 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. 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. 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. Healtheducation• Diet counseling for parents• Iron fortified formulas• Limitation of milk• Avoid beverages like tea• Colour changes of stool• Staining of teeth• Deworming
  • 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. Alleles• Homozygous-same identical alleles• Heterozygous- two different allelesAA, rrAr
  • 23. PATHOPHYSIOLOGY
  • 24. Change in molecularstability & solubilityTactoidsInsoluble fibre likestructuresDeformation of RBCVASOOCCLUSIONAdjacent Tissue Ischemia
  • 25. Clinical features
  • 26. VASO OCCLUSIVE CRISIS• ABDOMINAL PAIN• OBSTRUCTIVE JAUNDICE• HEMATURIA• PRIAPISM
  • 27. Clinical features
  • 28. ACUTE SPENIC SEQUESTRATION
  • 29. APLASTIC CRISIS• DIMINISHED PRODUCTION OF RBC• ACCELERATED RATE OF RBCDESTRUCTION• DUE TO BLOCKAGE OF MAJOR VESSELSHYPERHEMOLYTIC CRISISCVA
  • 30. Normal chest ray Acute chest syndrome
  • 31. Clinical features
  • 32. Diagnosis• New born screening• Chronic villus biopsy• Hb electrophoresis• Sickle turbidity test• Iso electric focusing• High performance liquid chromatography• Globin DNA analysis
  • 33. TreatmentMedical management• Supportive therapies• Symptomatic managementSurgical management• Spleenectomy• Surgical exploration of abdomen
  • 34. Medical managementOxygenationRestHydrationPain managementBlood transfusionAntibioticsGene therapyFolic acid supplementationElectrolyte replacement
  • 35. Alleviating Pain Warmth: increases blood flow Massaging and rubbing Hot fomentation Bandaging Rest Analgesics Cognitive Behavioral Therapy Relaxation therapy
  • 36. THALASSEMIAThalassemia is an autosomal recessive,characterized by decreased synthesis ofone of the polypeptide chains [ or ]α βthat form normal adult haemoglobinmolecule
  • 37. IncidenceMediterranean ancestry, blacks andSouthern china, South East Asia.In South East Asia it is 40 millionIn India its 50 %
  • 38. Main Types
  • 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. Causes
  • 41. Pathophysiology OfThalassemia
  • 42. Clinical features
  • 43. Contd..• PROMINENT FRONTALAND PARIETALBOSSING• MALOCCLUSION OFTEETH
  • 44. Contd….1. Pale skin or jaundice2. Protruding abdomen3. Enlarged spleen and liver4. Dark urine5. Fatigue
  • 45. diagnosis• Prenatal testing• Peripheral blood filmexamination• Complete blood count(CBC)
  • 46. • Hemoglobin studies.• Family genetic studies• Radiological findings-hair end appearance• NESTROFT TEST
  • 47. Blood transfusion• Type :saline washed packed redcells• Rate :10-15 ml/kg
  • 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. 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. Thank you….