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Anaemia

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Anaemia

  1. 1. ANAEMIA BY:- Saifuddin Lala M.B.A(Pharma)
  2. 2. DEFINATION[1]  Anaemia is define as reduction of the erythrocyte mass in the blood. In anaemia either the no. of erythrocyte are reduced or erythrocyte are deficient in the oxygen binding pigment haemoglobin.
  3. 3. INTRODUCTION[1] [2]  At the sea level, the average normal men has a 5.4 * 106 erythrocytes/mm3 & these erythrocytes contain 16 g of haemoglobin/dl of blood.  The volume occupied by the erythrocyte is 47% of the blood & is called as haematocreat. The haemoglobin level, below which the anaemia is likely to manifest are given below.
  4. 4. NORMAL HAEMOGLOBIN VALUE AGE AND SEX HAEMOGLOBIN LEVELE g/dl 1) 6 MONTHS-6YEARS 11 2) 6 YEARS-14 YEARS 12 3) MEN 13 4) WOMEN 12 5) PREGNANT WOMAN 11
  5. 5. HAEMOGLOBIN
  6. 6. GENERAL SYMPTOMS OF ANAEMIA
  7. 7. CONT...  There are approx 100 different types of anaemia few of them are classified as follow.  MYCROCYTIC ANAEMIS  Iron deficiency anaemia.  Anaemia of chronic disease.  Sideroblastic anaemia.  MEGALOBLASTIC ANAEMIAS  Float deficiency anaemia.  Vit.B-12 deficiency anaemia.
  8. 8. CONT...  HAEMOLYTIC ANAEMIAS  Auto immune haemolytic anaemia.  Sickle cell anaemia.  Thalassaemia.  Anaemia due to glucose-6-phosphat dehydroginase deficiency.  Paroxysmal nocturnal haemoglobinuria.
  9. 9. IRON DEFICIENCY ANAEMIA[3][4]  Iron is critical for the growth of all cells. It is therefore not surprising that iron-deficiency anemia independently increases morbidity and mortality. 1] IRON PROFILE IN BODY:-  Most adults have 45 mg/kg elemental iron in their bodies. Females generally have lower levels than males.  Within that pool of total body iron, approximately two thirds is contained in heme and one third in the storage forms of ferritin or hemosiderin.
  10. 10. CONT…  To maintain adequate supplies of iron for heme synthesis, 20 mg of iron is recycled daily, from senescent red cells that are removed from the circulation to new cells in the bone marrow.  Approximately 1 to 2 mg per day of additional dietary iron is needed to balance losses in urine, sweat, and stool.
  11. 11. MANAGEMENT[5]
  12. 12. MANAGEMENT[5]  Human erythropoietin can also be administrated. Transfusion should be prescribed ONLY for conditions for which there is NO OTHER TREATMENT.  Transfusion should not be prescribed when patient have congestive cardiac failure, chronic kidney disease, or liver dysfunction.  So in such kind of patients, we can administrated packed RBC.
  13. 13. ANAEMIA OF CHRONIC DISEASE[1]  A mild anaemia is often associated with chronic diseases. E.g. chronic infection, renal failure & rheumatoid arthritis etc.  The cause of anaemia is not well understood but adaptive physiological response to the inflammation may be responsible.
  14. 14. MEGALOBLASTIC ANAEMIA  Megaloblastic anemia is a blood disorder in which there is anaemia with larger than normal red blood cells[7]. SIGN AND SYMPTOM[6]  The anemia symptoms of both folic acid deficiency and vitamin B12 deficiency are the same. In both there are often gastrointestinal tract symptoms e.g. anorexia and diarrhea occur because the epithelial cells lining the intestines also have a rapid turnover.
  15. 15. SIGN AND SYMPTOM[6]  Frequently the patient does not seek medical help until some of the following conditions occur: weakness, palpitations, angina, and symptoms of congenital heart failure. Folic acid deficiency results in anemia and intestinal manifestations but not neurological symptoms.
  16. 16. CAUSES OF VITAMIN B AND FOLET DEFICIENCIES[8]
  17. 17. PATHOPHYSIOLOGY
  18. 18. LABORATORY INVESTIGATION
  19. 19. FURTHER INVESTIGATION Additional tests to establish the cause of low b12 concentration  Parietal cell and intrinsic factor antibodies: a positive result for intrinsic factor antibodies is diagnostic of pernicious anaemia as this is a more specific assay.  The Schilling test for B12 absorption is no longer available. Upper gastrointestinal endoscopy, when upper gastrointestinal symptoms or co-existing iron deficiency are present, chiefly to exclude gastric carcinoma, which occurs in 5% of patients with
  20. 20. FURTHER INVESTIGATION Additional tests to establish the cause of folate deficiency  In the absence of dietary deficiency (common in the elderly), a malabsorption syndrome (e.g. coeliac disease) should be excluded, particularly in patients with mixed iron and folate deficiency.  Coeliac screening estimation by detection of endomysial antibodies (EMA-IgA), anti-tissue transglutaminase (tTG IgA/IgG) and anti-gliadin (AGA IgA, AGA IgG) antibodies.  If the diagnosis is still in doubt, jejunal biopsy is the gold standard test.
  21. 21. FURTHER INVESTIGATION Bone marrow examination  It is rarely necessary in B12/folate deficiency, but should be done if assay values are inconclusive, particularly if the patient is pancytopenic, to exclude aplasia, myelodysplasia or neoplasia.  However, it is mandatory in other macrocytic conditions, especially to demonstrate the characteristic dyshaemtopoiesis of a myelodysplastic disorder.
  22. 22. MANAGEMENT:- B12 DEFICIENCY  Loading doses of hydroxocobalamin 1mg are administered I.M 3 times per week to a total of 6 doses in the first 2 weeks. Thereafter, it is given 3-monthly for life in cases of B12 malabsorption.  However, in dietary deficiency, maintenance oral supplementation with cyanocobalamin may be given once tissues stores have been replenished.  Blood transfusion should be avoided even in severe anaemia, unless symptoms or cardiovascular risks dictate otherwise.
  23. 23. MANAGEMENT:-  Neurological complications may develop even in the absence of anaemia, therefore it is essential to initiate treatment as soon as the diagnosis is made. Folic acid must not be initiated before B12 as this can precipitate or aggravate neurological complications. FOLATE DEFICIENCY  Folic acid is given at a dose of 5 mg daily orally for about 4 months to replenish tissue stores. Dietetic advice should be given if dietary deficiency is the cause. Prophylactic folate supplements, 400 μg daily, are recommended for pregnant
  24. 24. HAEMOLYTIC ANAEMIA  Haemolytic anaemia results from the early destruction of an erythrocyte, from a variety of causes, either inherited, or acquired. The mechanism may be via intravascular or extravascular damage. 1] SICKLE CELL DISEASE  Sickle cell disease (SCD) results from the recessive inheritance of a mutant β globin gene in which valine is substituted for glutamic acid at position 6 of the β -globin chain, resulting in the formation of
  25. 25. CONT’D  SCD comprises a group of conditions in which HbS is present either in the homozygous form, HbSS, or in association with other variant haemoglobins; HbC, HbE HbDPunjab, HbOArab, β-thalassaemia plus (β +) or β – thalassaemia zero (β0) leading to HbSC, HbSE and so on. PATHOPHYSIOLOGY:  Normal adult haemoglobin (HbA) is soluble whereas HbS is hydrophobic and has the singular property of
  26. 26. CONT’D  These bundles twist the shape of the red cell and damage its membrane leading to irreversibly sickled cells, which are targets for phagocytosis.  Sickle cells have lifespan of 16-20 days. Vasoocclusion by cells is not only case of sickle cells blocked microvasculature, but SCD is a chronic inflammatory condition with elevated conc. of inflammatory mediators and free radicals.  There is red cell dehydration and abnormal interaction with vascular endothelium & NO depletion, all contributing to vasoocclusion, the hallmark of sickle cell disease.
  27. 27. VASO-OCCLUSION
  28. 28. DIAGNOSIS AND LABORATORY FEATURES  Full blood count (FBC) will make known anaemia with a high reticulocyte count and blood film may show sickle cells. Serum bilirubin may be elevated as may serum lactate dehydrogenase.  The sickle solubility test quickly confirms the presence of sickle haemoglobin but is unable to differentiate unaffected carrier status from the disease.  The most widely used diagnostic test is HPLC, which separates haemoglobins according to their charge. Isoelectric focusing and mass spectrometry are used as alternatives in
  29. 29. MANAGEMENT  Exchange blood transfusion (EBT), where venesection is carried out simultaneously with transfusion to avoid raising the haematocrit, as this may cause hyperviscosity.  EBT can be carried out manually or by automated erythro-cytopheresis but is associated with a poorer outcome.  Hydroxycarbamide therapy is then commenced in patients with severe disease, aiming to improve it.  This results in increased HbF% and reduced white
  30. 30. CONT’D  Use of hydroxycarbamide is associated with reduced mortality and decreased frequency of painful crisis and acute chest crisis.  Allogeneic stem cell transplant is the only known cure for sickle cell disease and although over 200 transplants have been successfully carried out with less than 5% mortality rates worldwide.  Its use has been limited in adults by toxicity and the availability of a suitably fully human leukocyte antigen matched donors.  Recent successful transplants using 50% matched related donors and less toxic regimens9,10 offer much greater hope.
  31. 31. THALASSAEMIA  Thalassaemia is an inherited disorder of Hb synthesis where mutations of the globin gene lead to various degrees of quantitative defect in globin production and an imbalance in β globin chain synthesis, resulting in ineffective erythropoiesis and anaemia.  α-THALASSAEMIA  There are four a-globin genes, two inherited from each parent, loss of up to two genes does not result in a clinically apparent syndrome (permutations are; aa/a-,a-/a-,aa/–) however loss of three genes (a-/–) results in haemoglobin H disease, associated with anaemia and a degree of haemolysis.
  32. 32. CONT’D  These patients occasionally require transfusional support. Loss of all four genes results in death due to profound anaemia. β -THALASSAEMIA:  β-thalassaemia is a recessively inherited disorder where there is a reduction (b+) or absence (b0) of normal b- globin chains.  It is divided into three broad clinical phenotypes. Individuals with β-thalassaemia major are completely dependent on blood transfusion support from the first year of life, usually due to homozygous inheritance of 0
  33. 33. CONT’D  Thalassaemia minor (trait) patients inherit a normal b gene in association with either b+ or b0, and are asymptomatic but may have a mild anaemia with invariably low MCV and mean cell haemoglobin (MCH).  The third group is a heterogeneous group of patients with diverse phenotypes intermediate to thalassaemia major and minor, referred as thalassaemia intermedia. Pathophysiology  The anaemia in β-thalassaemia results from ineffective erythropoiesis, peripheral haemolysis and the overall reduction in haemoglobin synthesis.
  34. 34. PATHOPHYSIOLOGY  The reduced or absent β-globin production leads to precipitation of excess α-globin chains in red cell precursors within the haemopoietic tissue and results in their early destruction.  The effect of this ineffective erythropoiesis, a compensatory erythroid hyperplasia, expansion of haemopoietic tissue within expanded marrow spaces, extramedullary haemopoiesis & hepatosplenomegaly in the absence of adequate transfusion.  These patients also have markedly increased iron absorption, made worse with transfusional iron overload, and the condition leads to early mortality if untreated.
  35. 35. CLINICAL INVESTIGATION & MANAGEMENT  CBC confirms a microcytic & hypochromic anaemia, and blood film shows red cells that are nucleated and have basophilic stippling.  HPLC will reveal an absence of HbA in thalassaemia major, raised haemoglobin A2 in thalassaemia intermedia patients with variable amounts of HbA and HbF, & raised haemoglobin A2 in thalassaemia trait.  children with untreated or partially treated thalassaemia major die in the first or second decade of life. Clinical management is based on adequate, safe blood transfusions & prevention of iron overload.
  36. 36. REFERENCES 1] Herfindal ET, Gourley DR, Hart LL. ‘clinical pharmacy & theraputics’ walter kluwer health 2011: 57-90 2] Walker R, Whittlesea C ‘clinical pharmacy & theraputics’ published by churchil livingstone elsevier; 2008: 769-785. 3] Heeney MM, Andrews NC. Iron homeostasis and inherited iron overload disorders: an overview. Hematol Oncol Clin North Am 2004;18:1379-1403. 4] Kemna EH, Tjalsma H, Willems HL, Swinkels DW. Hepcidin: from discovery to differential diagnosis. Haematologica. 2008;93:90-97. 5] Michael Alleyne , McDonald K. Horne , Jeffery L. Miller: Individualized Treatment for Iron-deficiency Anemia in Adults The American Journal of Medicine Volume 121, Issue 11 2008 943 – 948.
  37. 37. REFERENCES 6] Braunwald E, et al., eds., Harrison’s principles of Internal Medicine.15thed. New York, McGraw-Hill. 2001; 674-680 7] Nissenson AR, Goodnough LT, Dubois RW. Anemia: or just an innocent bystander? Arch Intern Med. 2003;163:1400-1404. 8] Wallerstein RO Laboratoryevaluation of anemia. WestJ Med 1987Apr; 146:443-451 9] Reynolds EH. Benefits and risks of folic acid to the nervous system. J Neurol Neurosurg Psychiatry 2002; 72: 567–71. 10] BolanosMJ, Fuchs EJ, Luznik L, et al. HLA-haploidentical bone marrow transplantation with post-transplant cyclophosphamide expands the donor pool for patients with sickle cell disease. Blood 2012; 120: 4285 91. 11] Modell B, Darlison M. Global epidemiology of haemoglobin disorders and derived service indicators. Bull World Health Organ 2008; 86: 480 7

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