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

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

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

28. Clinical features

31. VASO OCCLUSIVE CRISIS
• ABDOMINAL PAIN
• OBSTRUCTIVE JAUNDICE
• HEMATURIA
• PRIAPISM

32. Clinical features

33. ACUTE SPENIC SEQUESTRATION

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

36. Clinical features

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

48. Pathophysiology Of
Thalassemia

50. Clinical features

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….