Anemia is defined as a reduction in red blood cells or hemoglobin levels below normal. It can be caused by nutritional deficiencies like iron deficiency, impaired red blood cell production like in megaloblastic anemia, or genetic disorders such as sickle cell anemia, thalassemia, and hereditary spherocytosis. Treatment depends on the underlying cause but may include oral or intravenous iron supplementation, vitamin B12 or folate, blood transfusions, or managing symptoms during sickle cell crises.

1. ANEMIA
(91-95)

2. DEFINITION
• Anemia is defined as reduction of the total circulating red cell mass
below the normal limits which reduces the oxygen carrying capacity
of blood which leads to tissue hypoxia.
• Anemia is usually diagnosed based on a reduction in the Hematocrit
(the ratio of packed red cells to total blood volume) and the
haemoglobin concentration of the blood to levels that are below the
normal range according to the age and gender of the individual.

4. Erythropoiesis
• It is a regulated process of red cell production.

5. PHYSIOLOGICAL CLASSIFICATION OF ANEMIA

6. MEGALOBLASTIC ANEMIA
• Anemia is due to impaired DNA synthesis resulting in delayed mitosis
where RNA and protein synthesis is not impaired.
• The RBCs are large in size but immature.
• There is ineffective erythropoiesis and macro-ovalocytes are found on
peripheral smear, along with Hyprsegmented PMNLs.
• The bone marrow is usually hypercellular.
• Nucleocytoplasmic asynchrony is present.

8. IRON DEFICIENCY ANEMIA
• Most common cause of nutritional anemia worldwide.
• SERUM Iron levels decrease below normal levels( 50-150
micrograms/dL).
There are 3 stages of iron deficiency-
-Negative iron balance
-Iron deficient erythropoiesis
-Iron deficiency anemia

11. HEREDITARY SPHEROCYTOSIS
• It is an autosomal dominant disorder.
• Normally ,RBC membrane consist of a protein Spectrin and Ankyrin.
• Mostly the mutation in Ankyrin.
• Spectrin defect in hereditary elliptocytosis.

12. Pathophysiology

13. SICKLE CELL ANEMIA
• It is autosomal recessive disease in which there is point mutation at
the 6th position of beta globin chain where glutamic acid is replaced
by valine residue.
• This mutation leads to the formation of Hb S haemoglobin.
• Factors causing sickling :
- Hypoxia and oxidative stress
- Decrease in ph
- Intracellular dehydration
• Factor decreasing sickling : Fetal haemoglobin

15. THALASSEMIA
• It is a Group of autosomal recessive inherited chromosomal disorder
characterized by decreased synthesis of either alpha or beta haemoglobin.
• Beta thalassemia is of 3 types:
-thalassemia major
-thalassemia intermedia
-thalassemia minor
• Alpha thalassemia is of 4 types:
-silent carrier
-thalassemia trait
-HbH disease
-hydrops fetalis

16. G-6-PD DEFICIENCY
• X-linked recessive disorder.
• Deficiency of Glucose 6 Phosphate dehydrogenase enzyme deficiency
that reduces the ability of red cells to withstand oxidative injury.
• Conditions causing this-
- Infections
- Drugs- antimalarial drugs ,sulphonamides
- FAVA beans

17. CLINICAL PRESENTATION OF ANEMIA
SYMPTOMS:
• Easy fatigability and weakness
• Malaise and lethargy
• Shortness of breath and headache
• Dizziness and difficulty in concentration
• Leg cramps
• Palpitation
• Impaired libido

18. In severe anaemia,
• Fainting
• Chest pain
• Angina
• Heart attack
• Worsening CHF

19. SIGNS OF ANEMIA
• Pallor
• Brittle hair and nails
• Koilonychias
• Angular stomatitis
• Dysphagia and glossitis
• Atrophy of papilla of tongue
• Plummer Vinson Syndrome
• Atrophic glossitis

20. • Angular cheilosis
• Jaundice
• Skin hyperpigmentation

21. Laboratory evaluation

23. APPROACH

25. Severe Iron deficiency
anemia
Anisocytosis (size), Poikilocytosis (shape)
Normal peripheral smear

26. Thalassemia
• Haemoglobin reduced
• TLC and platelet normal
• MCV <80 ,MCH <27 ,MCHC normal
• HbF increase on HB electrophoresis
• Qualitative and quantitative estimation HBF by APT test and Keilhauer test
respectively
• HbA2 increase (3.5 -9%) characteristic of Thalassemia minor
• NESTROF test screening
• MENTZER index: MCV / RBC count :FOR IDA (>13) ,for thalassemia minor
<13
• On bone marrow aspiration : hypercellular and reversal of M:E ratio

27. Thalassemia
Target cells

28. Megaloblastic anemia
• Haemoglobin , platlet , total lecocyte count decrease
• Retic count decrease ,MCV increase ,MCHC normal
• Serum cobalamine (160-1000ng/lt) level less than 100ng/lt
• Serum methyl malonate and homocysteine level raised
• Serum folate level decrease (2-15 micro/dl)
• Serum antibodies against intrinsic factor
• Shilling test
• On bone marrow aspirate : hypercellular , M:E ratio reversal ,increase
no. of immature erythroid precursor

29. Howell-Jolly bodies

30. Sickle cell anemia
• Haemoglobin decrease
• TLC and platelet count normal
• Retic count increase
• Sickling test : sickling is induced by 2 % Metabisulphite
• Sickle cell band on Hb electrophoresis
• High performance liquid chromatography to quantify different Hb
• HbF estimation
• Parental genetic testing

32. Hereditary spherocytosis
• Haemoglobin decrease , TLC and platelet count normal
• MCHC increase and retic count increase
• Osmotic fragility test and pink test
• Flow cytometric evaluation of RBC membrane protein

33. G6PD deficiency
• Haemoglobin decrease , TLC , platelet normal
• Retic count normal
• Methylene blue reduction test
• Heinz bodies and bite cell on peripheral smear

34. Aplastic Anemia
• Hb , platelet, TLC , Reticulocyte count decrease.
• MCV, MCH, MCHC normal.
• On Bone marrow aspiration, a “dry tap” is found.
• On biopsy, fat spaces are increased.

36. MANAGEMENT

37. Iron Deficiency Anemia
1.Oral iron therapy –
• Given in asymptomatic patients with established iron deficiency.
• ferrous sulphate 325 mg tds (65 mg elemental iron)
• Dose of 200 mg of elemental iron per day result in absorption of upto 50 mg/day
• Other preparations –
• Ferrous fumarate
• Ferrous gluconate
• Polysaccharide iron

38. Parenteral Iron Therapy
• Indications-
• Patients who are unable to tolerate oral iron
• If the needs are acute
• Those who need iron on an ongoing basis ( e.g. persistent GI blood loss)
• Available preparations-
• Iron sucrose (venofer)
• Sodium ferric gluconate (ferrlecit)

39. • Calculation of iron deficit-
• Body weight(kg) * 2.3 *(15- pt.s Hb) + 500 or 1000 ( for stores)
• Either total dose of iron is infused on a single infusion or
• Repeated IM injections often over a protracted period
• Injections are given with z technique

40. 3.Red Cell Transfusion
• This is reserved for individuals who have symptoms of anemia ,
cardiovascular instability and continued excessive blood loss and
require Immediate intervention.
• Once the patient is stabilized , other options can be renewed.

41. Megaloblastic Anemia
• Cause of the anemia has to be established ( whether folate or
cobalamin )
• COBALAMINE deficiency
• Underlying cause for the deficiency can be corrected in some cases
(e.g. fish tapeworm , tropical sprue ,intestinal stagnant loop)
• Available preparation
• Hydroxy cobalamin
• Cyano cobalamin
(replenishment of body stores usually complete with six 1000 microgm IM
injections of hydroxycobalamin given at 3to7 day intervals and for maintenance
one injection every 3 months)

42. • FOLATE deficiency
• Daily Oral dose of 5-15 mg folic acid carried for usually 4 months is
satisfactory .
• Long term therapy is required when underlying cause cannot be
corrected (e.g. chr. Dialysis , hemolytic anemias)
• Cobalamin deficiency must be excluded.
• Other preparation
• Folinic acid

43. Sickle Cell Anemia
• These patients require ongoing continuous care .
• These include
• Regular slit lamp examination to monitor retinopathy.
• Antibiotic prophylaxis for patients after splenectomy.
• Vigorous oral hydration during extreme exercise , exposure to heat and cold
,emotional stress
• During acute crisis
• Vigorous hydration and aggressive analgesia (with morphine or ketorolac)
• Blood transfusion is reserved for extreme cases.
• Bone marrow transplantation is effective and safe only in children.

44. Thalassemia
• For thalassemia major
• Patients require chronic hyper transfusion therapy to maintain a hematocrit
of 27-30% , so that erythropoiesis is suppressed .
• Splenectomy is required in patients if annual transfusion requirement
increases by >50%.
• For thalassemia minor
• These patients donot require transfusion.
• Empirical use of iron is recommended.

45. Aplastic Anemia
• Either replacement of absent hematopoietic cells
• Best therapy for younger patients with a full histocompatibility donor.
• Suppression of immune system to allow recovery with residual bone
marrow of patient.
• Anti-thymocyte globulin in combination with cyclosporine
• Response is better in children
• Relapse pancytopenia is a frequent problem as cyclosporine is discontinued.

46. Hereditary Spherocytosis
• No treatment is available.
• Splenectomy is done in severe cases.
• G-6-D deficiency
• Acute hemolysis is largely preventable by avoiding exposure to
triggering factors like consumption of fava beans , certain drugs like
anti malarials, sulphonamides.
• If anemia is severe , may require immediate action including blood
transfusion.

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