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Specific Anemias.ppt
- 2. Iron Deficiency Anemia(IDA) Anemia of Chronic Disease(ACD) Megaloblastic Anemia Hemolytic Anemia Other Types Aplastic Anemia(AA) Anemia of CRF
- 3. Introduction Epidemiology Iron Metabolism Causes Clinical Features Diagnosis Differential Diagnoses Treatment
- 4. The development & the rapidity of IDA is dependent upon the body’s iron store. Iron store in turn depends on ◦ Age ◦ Sex ◦ Rate of growth ◦ Balance b/n loss & absorption Generally lower Iron values for women because of menstrual loss, pregnancy, lactation
- 5. IDA is supposed to be one of the commonest forms in our country 1 - 2% even in the Western countries Iron deficiency without anemia 11% in women (men 4%) The WHO data on anemia 1997 2 billion people affected Children & pregnant women most affected Prevalence Young children 48% Pregnant women51% Non-pregnant women 35% Adult men 18%
- 6. On IDA ◦ Commonest among the nutritional anemias ◦ More prevalent in developing countries 36% vs 8% Local data show similar pictures ◦ North Western Ethiopia General population 40.5% Children 47.2% ◦ Jimma 57% has been reported in pregnant women
- 7. Regulation of iron balance ◦ Interaction of various proteins ◦ Interplay b/n iron absorption & loss
- 8. Transferrin (Tf) & its receptors (TfR) Ferritin Iron responsive element-binding protein( IRE-BP), IRP/IRF HFE Divalent Metal Transporter( DMT1) Stimulator of iron transport(SFT) Ferroportin & Hephaestin Hepcidin
- 9. Transports Fe3+ through plasma Synthesized in the liver Increased production in deficiency states Measured as Tf or TIBC 1/3rd saturated normally Fe/Tf = 33%
- 10. States with decreased Tf saturation or increased TIBC IDA ACD ( occasionally) Ferroportin mutation States with increased Tf saturation or decreased TIBC Aplastic anemia Sideroblastic anemia Ineffective erythropoiesis hemochromatosis
- 11. Huge molecule for cellular storage of Fe ◦ 4500 atoms of iron Acute phase reactant Accessible for metabolic needs Excess is changed to Hemosiderin ◦ Hemosiderin is not easy accessible for metabolism Measured as apoferritin in the plasma Plasma level reflects body iron store ◦ 1ng of ferritin= 10mg of total iron store
- 12. Normal body content = 3-4gm HGB = 2.5gm Other Fe containing proteins= 400mg Tf bound in the plasma= 3-7mg The rest as ferritin/hemosiderin= body Fe store ◦ Adult men = 1gm ◦ Adult women = much less than men Daily iron loss = 1mg
- 13. Diet contains heme & non-heme Fe ◦ 30% vs 10% absorbed Released in the acidic environment and is sent with mucin to the doudenum for absorption Enhancers vs Inhibitors Ferric vs ferrous
- 17. Blood loss( major cause) ◦ Obvious or occult ◦ GI loss the main, including Hook worm Decreased absorption( uncommon) ◦ Part of generalized malabsorption like Celiac & Tropical Sprue
- 18. Others causes ◦ Pulmonary hemosiderosis ◦ Intravascular hemolysis Hemoglobinuria & hemosiderinuria ◦ Increased demand Rx with EPO, folate or Vitamin B12
- 19. IDA has 3stage Stage 1 ◦ Depleted iron store without anemia ◦ High risk of anemia with slightest bleeding ◦ Iron from daily RBC turn over
- 20. Stage 2 ◦ Normocytic ◦ Normal reticulocyte count ◦ Common in developed countries
- 21. Stage 3 ◦ Typical character ◦ In the face severe Fe deficiency ◦ Microcytic hypochromic ◦ Imbalance b/n heme & globulin synthesis High EPO LOW RETIC COUNT
- 23. Features of anemia ( as discussed) Typical of IDA ◦ KOILONYCHIA( SPOON NAILS) ◦ BLUE SCLERA ◦ PICA & PAGOPHAGIA ◦ BEETURIA ◦ PATERSON-KELLY/PLUMMER-VINSON SYNDROME Dysphagia Oesophageal web Atrophic glossitis
- 24. Low HGB, HCT, RBC counts Microcytic-hypochromic ◦ Low MCV, MCH &MCHC ◦ Increased central pallor High Platelet count( Reactive thrombocytosis) Normal WBC Count & Morphology
- 25. Low serum iron & ferritin High Tf level ◦ Low saturation ( 2.5%) ◦ High TIBC Absent iron stores Brisk response to therapeutic trail
- 27. The dx of IDA has 2 components ◦ Confirmation of iron deficiency ◦ Identify the cause
- 28. CBC &ESR RBC INDICES PERIPHERAL SMEAR IRON STUDIES ◦ Serum iron ◦ Serum ferritin ◦ Serum Tf, TIBC BM STUDY THERAPEUTIC TRAIL & OTHERS
- 29. History is vital ◦ Gynecologic ◦ History of surgery ◦ Dietary habit Stool exam ◦ Occult blood ◦ Ova of parasites Endoscopy colonoscopy
- 30. When anemia is mild with normal indices (Stage 2 IDA) ◦ ACD ◦ Anemia due to CRF ◦ Anemia due to endocrine disorders Classic(microcytic- hypochromic) ◦ Thalassemia ◦ Sideroblastic anemia ◦ ACD ◦ Lead poisoning
- 31. Two aspects ◦ Treatment of the underlying cause ◦ Administration of iron
- 32. General principles of iron treatment Iron is absorbed in the duodenum & pro.jejunem Shouldn’t be given with food Best absorbed in a mildly acidic media Give ascorbic acid 2hrs before or 4hrs after antacids Cost and effectiveness UGI discomfort is directly related to the amount of iron Try the elixir
- 33. Oral preparation ◦ Ferrous sulfate 65mg of elemental iron ◦ Ferrous fumarate 106mg of elemental iron ◦ Ferrous gluconate 28-36mg of elemental iron ◦ Ferrous sulfate elixir 44mg/5ml Side effect ◦ GI upset
- 34. 150-200mg of elemental iron Response to treatment ◦ Reticulocytosis in 7days ◦ Rise in HGB 2g% over 3weeks ◦ Reasons for failure Duration of treatment ◦ Continue after normalization of HGB to replenish the iron stores ◦ 3-6months after normalization of HGB
- 35. Parenteral iron ◦ IV or IM ◦ Preparations Iron dextran Ferric gluconate Iron sucrose ◦ Major side effects Local & systemic
- 37. Also called anemia of chronic inflammation ACD can be associated with conditions other than inflammation, infection or malignancy: Severe trauma Heart disease Diabetes Acute or chronic immune reactivation
- 39. Primarily reflect a reduction in RBC production but there may be a component of RBC reduced survival
- 40. Three factors for hypoproliferative state ◦ Trapping of iron in macrophages ◦ Reduced sensitivity of BM to EPO Normal marrow Increase apoptosis of RBC precursors ◦ Relative reduction in EPO
- 41. Release of cytokines ◦ Interleukins ◦ TNF ◦ Interferon ◦ Hepcidin
- 42. Acute variant ◦ “Anemia of critical illness” ◦ Acute event related Major surgery MI Sepsis Major trauma
- 43. Anemia in ACD is of variable severity ◦ Many 10-11g% hgb ◦ Some(20%) will have HGB of 8g% or less Low absolute reticulocyte count High acute phase reactants & cytokines Low serum iron & Tf level(TIBC)
- 44. 1/4th of patients are iron deficient Serum ferritin normal or elevated Bone marrow ◦ Macrophages increased or normal iron content ◦ Erythroid precursers – decreased or absent
- 45. ACD is usually NC & hypoproliferative ◦ CRF ◦ Severe endocrine disorders
- 46. Some patients with severe anemia & microcytic hypochromic picture ◦ IDA ◦ THALASSEMIA ◦ SIDEROBLASTIC ANEMIA
- 47. Usually mild & may not interfere with quality of life Correction of underlying problem Erythropoietin ◦ After measuring the level ◦ ?Survival ◦ Darbepoetin Supplemental iron ◦ To maintain 20% or above saturation of Tf
- 48. INTRODUCTION CLINICAL PRESENTATION LABORATORY FINDINGS & DX TREATMENT
- 49. Megaloblasts are nucleated RBC precursors in the BM which maybe seen in rarely in severe deficiency states Macrocytic anemia is more appropriate Anemia caused by vitamin B12(cobalamin) deficiency and folate deficiency is similar
- 50. Vitamin B12 deficiency ◦ Take longer time to develop ◦ Neurological manifestations may come early Folate deficiency ◦ Can develop in relatively short duration Pregnancy Acute & severe infection Severe hemolysis
- 54. Classic presentation ◦ Anemia with MCV > 100fl(usually >115) ◦ Low –normal or low ARC ◦ Macro-ovalocytes ?megaloblasts ◦ Hypersegemented neutrophils
- 55. Masked by iron deficiency/ thalassemia Neurologic manifestation ◦ Cyanobalamin ◦ Without anemia Only hypersegemented neutrophils
- 57. Evaluation has two stages ◦ Deficiency state ◦ Cause History very important CBC Peripheral smear Serum Cbl level RBC folate level
- 58. Specific metabolites ◦ Elevated methylmalonic acid(MMA) ONLY IN CBL def ◦ Elevated homocysteine Both folate & Cbl deficiency BM Other tests ◦ Schilling test( 3 stages) ◦ Antibodies Intrinsic factor Parietal cell Empiric treatment in the case of folate deficiency
- 60. Folate deficiency ◦ 1-5mg/day oral ◦ For 1-4months ◦ Complete hematologic recovery Reduction in LDH in 2days Reticulocytosis in 3-4days(peak in 1wk) Rise in HBG & reduction in MCV in 8wks Delay in cases of iron deficiency
- 61. Cobalamine deficiency ◦ IM CBL 1000microgram(1mg) daily for 1wk 1mg/wk for 1month 1mg/month then after Cause not known or PA- give for life monthly
- 63. HA ◦ Vast group ◦ Important cause of anemia ◦ Shorted RBC survival ◦ Splenomegaly(hypersplenism) Hemolysis ◦ Shorted RBC survival less than 100days ◦ Normal RBC survival- 120days ◦ Not necessarily HA
- 64. Random hemolysis ◦ Another way of RBC destruction besides the death of aging (senescent) RBC ◦ Age independent ◦ Very low rate Less than 0.05-0.5% Increased in the case of splenomegaly & HA ◦ Well compensated normally by Increased EPO production Reticulocytosis
- 65. RBC lifespan & turnover ◦ Estimated from Reticulocyte percent HCT Reticulocyte life span ◦ Formula RBC survival (days) = 100/[retic%/RLS(days)] RBC turnover(%/d) = 100/RBC survival (days)
- 67. Absolute reticulocyte count ◦ Reticulocyte percent may not give all the information about the BM response ◦ Normal value 25,000-75,000/microL ◦ Can be counted ◦ Corrected Corrected ARC= ARC/retic maturation time More informative Reticulocyte production index(RPI) ◦ Two corrections Survival shift Degree of anemia ◦ Formula RPI= retic% x (HCT/45) x ½ Normal 1
- 68. Different ways of classification of causes of HA ◦ Intracorpuscular/ Extracorpuscular ◦ Inherited/ Acquired ◦ Intravascular/ extravascular ◦ Immune-mediated/ non-immune mediated Immune mediated Warm/ cold Antibody
- 70. Starts with an accurate hx & P/E Classic case ◦ New onset of pallor or anemia ◦ Jaundice ( high indirect bilirubin) ◦ Gallstones ◦ Splenomegaly ◦ Presence of circulating spherocytic RC ◦ Increased LDH ◦ Decreased serum haptoglobin ◦ + Coomb’s test ◦ High retic % or ARC
- 71. Peripheral smear ◦ Spherocytes ◦ Fragmented RBC ◦ Acanthocytes (spur cell) ◦ Teardrop cell ◦ Blister or “ bite” cells ◦ RBC inclusions ◦ Parasites
- 72. Other forms of anemia ◦ Aplastic amemia Idiopathic Acquired ◦ HA AIHA ◦ ANEMIA OF CRF