Anemia Anak 2


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Anemia Anak 2

  1. 1. Anemia Anak 2 dr. Bertha
  2. 2. Physiologic Anemia of Infancy <ul><li>Normal newborn -> higher Hb & Ht, larger RBC </li></ul><ul><li>Within 1 st week  progressive decline in Hb level begins and persists for 6-8 weeks  Physiologic anemia </li></ul><ul><li>Normaly reaches between 8-12 weeks old (Hb 9-11 g/dl) </li></ul>
  3. 3. Physiologic Anemia of Infancy <ul><li>Cause </li></ul><ul><ul><li>Decrease EPO production </li></ul></ul><ul><ul><li>Switch from fetal to adult Hb </li></ul></ul><ul><ul><li>Frequent blood sampling in preterm infant </li></ul></ul><ul><ul><li>Short RBC life span </li></ul></ul><ul><ul><li>Rapid growth </li></ul></ul>
  4. 4. Physiologic Anemia of Infancy <ul><li>Treatment </li></ul><ul><ul><li>Ensuring that the diet contains essential nutrients for hematoposis (folic acid & iron) </li></ul></ul><ul><ul><li>Transfusion </li></ul></ul><ul><ul><li>FPO </li></ul></ul><ul><ul><li>Complemental iron </li></ul></ul>
  5. 5. Facts!! <ul><li>Iron absorbed in proximal small intenstine mediated by duodenal protein (HFE, mobilfernin, hephaestin) </li></ul><ul><li>Iron absorbed 2-3x more efficiently from human milk than cow’s milk </li></ul><ul><li>Distribution of iron in the body: </li></ul><ul><ul><li>Circulating RBC, muscle protein myoglobin </li></ul></ul><ul><ul><ul><li>12% : Iron storage protein </li></ul></ul></ul><ul><ul><ul><li>… % :….. </li></ul></ul></ul>
  6. 6. Facts!! <ul><li>Breast fed Infants should receive iron supplement from 4 month of age </li></ul><ul><li>During the first 2-3 month (physiologic anemia of infancy)  iron reclaimend and store  enough for blood formation in first 6-9 month f age </li></ul><ul><li>Anemia caused by un adequate duetary iron  commoning 9-24 month of age </li></ul><ul><li>Chronic iron deficiency anemia, causes: </li></ul><ul><ul><li>Lession in GIT (peptic ulcer’s polyyp, Meckel diverticulum, hemangioma, hookworm infestation) </li></ul></ul><ul><ul><li>Choronic diarhea </li></ul></ul>
  7. 7. Iron Deficieancy Anemia
  8. 8. Stage of Iron Deficieancy Anemia <ul><li>1. Depletion of iron store  no functional changes </li></ul><ul><li>2. Iron store exhausted  Tissue begin to have insufficient iron  iron deficiency </li></ul><ul><li>Outright anemia isn’t yet detected, but this deficiency will impair kognitive, ↓physical capacity,↓ imunity </li></ul><ul><li>3. Iron deficiency anemia </li></ul><ul><ul><li>- Hb 7-9 g/dl : moderate anemia </li></ul></ul><ul><ul><li>- Hb < 7 g/dl : Severe anemia </li></ul></ul>
  9. 9. Clinical Manifestation <ul><li>Most importang sign : Pallor </li></ul><ul><li>Iron deficiency  Effect on neurologic and intelectual function (attention span, alertness, learning) </li></ul><ul><li>When Hb <5 g/dl: </li></ul><ul><ul><li>Irrittability, anorexia, tachycardia,cardiac dilatation, systolic murmurs </li></ul></ul>
  10. 10. Progessive Iron deficiency <ul><li>Tissue iron store (bone marrow hemosiderin) disapper  serum fernitin level ↓  serum iron level ↓  Iron binding capacity↑  Transferrin saturation ↓ below normal  availability of iron for Hb synthesis ↓  free erythrocyte protoporphyrin (FEP) ↑ </li></ul><ul><li>RBC : microcytosis, hypochromia, poikilocytosis and increase RDW </li></ul><ul><li>Reticulocyte normal or decrease </li></ul><ul><li>Thrombocytosis my occur (some struktural homology between ertyhopoeitin and thombopoeitin). Few cases will have thrombocytopenia (in very severe iron deficiency anemia) </li></ul><ul><li>Bone marrow hypercellular with erythroid hyperplasia </li></ul>
  11. 11. Laboratory Finding <ul><li>Hb </li></ul><ul><ul><li>Essential for diagnosis of anemia, easiets, less expensive </li></ul></ul><ul><ul><li>Not very sensitive and specific for iron deficiency (only the 3 rd stage affects Hb synthesis) </li></ul></ul><ul><li>Ferritin </li></ul><ul><ul><li>Currently considered the most important indicator </li></ul></ul><ul><ul><li>COncentrartion decrease even un the 1 st stage of iron deficiency </li></ul></ul><ul><ul><li>Most sensitive indicator </li></ul></ul><ul><ul><li>Influenced by nany factor : infection, inflamation ↑ </li></ul></ul>
  12. 12. Laboratory Findings <ul><li>Soluble Transferrin Receptor (sTfR) </li></ul><ul><ul><li>Incresingly being used to determine iron deficiency where infection is a factors </li></ul></ul><ul><ul><li>Not as sensitive as ferritin, but more sensitive than Hb </li></ul></ul>
  13. 13. Differential Diagnosis <ul><li> - and β - thalasemia trait </li></ul><ul><li>Lead poisoning anemia </li></ul><ul><li>Chronic inflamation of infection usually normocytic, but may be slightly microcytic. Serum iron level and iron binding capacity reduced, serum ferritin normal or elevated </li></ul>
  14. 14. Treatment <ul><li>Oral administration of simple ferrous salts (sulfate, gluconate, fumarate) </li></ul><ul><li>Therapeutic does: elemental iron 4-6 mg/kg/day on 3 divided dose </li></ul><ul><li>Intolerance to oral iron theraphy is uncommon in young children. Older children and adolescent  GI complaints </li></ul><ul><li>Education re:diet </li></ul><ul><li>Parental iron preparation (iron dextran) is usually safe </li></ul><ul><li>Respone to parenteral iron is no more rapid or complate than that obtained with proper oral administration of iron </li></ul>
  15. 15. Treatment <ul><li>Medication should be continue for 8 week after blood value are normal </li></ul><ul><li>Blood transfusion indocated only when the anemia is very severe </li></ul>
  16. 16. Respone to iron therapy Time after Fe administration Respone 12-24 hr Replacement of intracellular Fe enzymes, decrease irritability, increase appetite 36-48 hr Initial bone marrow response, erythroid hyperplasia 48-72 hr Reticulcytosis, peaking at 5-7 days 4-30 hr Increase in Hb level > 1 month Repletion of tore
  17. 17. Anemia Hemolitic
  18. 18. Definition <ul><li>Premature destruction of RBC </li></ul><ul><li>If the rate of destruction exceeds the capacity of marrow to produce RBC  Anemia </li></ul><ul><li>Hemolytic process  EPO released  increase marrow activity  reticulocytosis </li></ul><ul><li>↑ Hb degradation  ↑ biliary excretion  ↑ fecal urobilinogen </li></ul>
  19. 19. Classification <ul><li>Intracellular </li></ul><ul><ul><li>Membrane abnormality </li></ul></ul><ul><ul><ul><li>Hereditary spherocytosis, eliptocytosis, stomatocytosis </li></ul></ul></ul><ul><ul><li>Enzyme deficiency </li></ul></ul><ul><ul><ul><li>G6DP deficiency </li></ul></ul></ul><ul><ul><li>Hemoglobinopathy </li></ul></ul><ul><ul><ul><li>Thalasemia </li></ul></ul></ul>
  20. 20. Clasification <ul><li>Extracellular </li></ul><ul><ul><li>Antobodies/autoimune </li></ul></ul><ul><ul><ul><li>Autoimune hemolitic anemia </li></ul></ul></ul><ul><ul><li>Mechanical factor </li></ul></ul><ul><ul><ul><li>Hypersplenism </li></ul></ul></ul><ul><ul><li>Plasma factor </li></ul></ul><ul><ul><ul><li>Liver Disease </li></ul></ul></ul><ul><ul><ul><li>Infection </li></ul></ul></ul>
  21. 21. Thalassemia <ul><li>Most common genetic disorder worldwide </li></ul><ul><ul><li>Shorter RBC life span </li></ul></ul><ul><ul><li>Fetal Hb </li></ul></ul><ul><ul><li>RBC more sensitive to oxidatives stress </li></ul></ul><ul><li>More than 200 mutation </li></ul><ul><li>3% of world population carry genes for β thalasemia </li></ul><ul><li>In South East Asia  5-10% of the population carry genes for  thalassemia </li></ul>
  22. 22. β Thalassemia <ul><li>β Thalassemia: excess  -globin relative to β &  globin chains </li></ul><ul><li> 2 β 2 (HbA)   4 atau  2  2 (HbA2) atau  2  2 (HbF) </li></ul><ul><li> Thalassemia : fewer  -globin chain </li></ul><ul><li> 2 β 2 (HbA)   4 (Bart’s Hb) atau  4 (HbH) </li></ul>
  23. 23. <ul><li>According to the degree of anemia: </li></ul><ul><ul><li>Thalassemia trait </li></ul></ul><ul><ul><li>Minima </li></ul></ul><ul><ul><li>Minor </li></ul></ul><ul><ul><li>Intermedia </li></ul></ul><ul><ul><li>Major </li></ul></ul><ul><li>Genetic classification doesn’t define the phenotype </li></ul><ul><li>Degree of anemia doesn’t predict the genetic clasification </li></ul>β Thalassemia
  24. 24. <ul><li>Thalassemia intermedia (heterozygotes thalassemia) </li></ul><ul><ul><li>Microcytic anemia </li></ul></ul><ul><ul><li>Hb about 7 g/dl </li></ul></ul><ul><ul><li>Extramedullary hematopoiesis </li></ul></ul><ul><li>Thalassemia minima & minor (heterozygotes thalassemia): </li></ul><ul><ul><li>Phenotype more severe that trait, not as severe…… </li></ul></ul>β Thalassemia
  25. 25. <ul><li>Thalassemia trait: </li></ul><ul><ul><li>Frequently misdiagnosis as iron deficiency </li></ul></ul><ul><ul><li>Short course iron therapy & evaluation  to separate patien who will need further evaluation </li></ul></ul><ul><ul><li>Silent form of thalassemia </li></ul></ul>β Thalassemia
  26. 26. Homozygous β o Thalassemia (Cooley’s anemia) <ul><li>Progessive hemolytic </li></ul><ul><li>Profound weakness & cardiac decompensation after 6 month old if not treated </li></ul><ul><li>Thalassemic facies </li></ul><ul><li>Pathologic fracture </li></ul><ul><li>Hepatosplenomegaly </li></ul><ul><li>Cachexia </li></ul><ul><li>Expanded medullary space </li></ul><ul><li>Iron over </li></ul>
  27. 27. Lab Finding <ul><li>Hb Electrophoresis </li></ul><ul><li>Anemia </li></ul><ul><li>Reticulocytosis </li></ul><ul><li>Numerous nucleated RBC </li></ul><ul><li>Microcytosis </li></ul><ul><li>↑ Unconjugated bilirubin </li></ul><ul><li>↑ Serum transferin </li></ul>
  28. 28. Treatment <ul><li>Blood transfusion </li></ul><ul><li>Iron chelation (desferoxamine) </li></ul><ul><li>Splenectomy </li></ul><ul><li>Bone marrow transplantation </li></ul>
  29. 29.  Thalassemia <ul><li>Most common is South East Asia </li></ul><ul><li>↑ Production of Bart’s Hb (  4) </li></ul><ul><li>Deletion mutation </li></ul><ul><li>Need PCR for diagnosis </li></ul>
  30. 30.  Thalassemia <ul><li>Deletion of 1  globin gene </li></ul><ul><ul><li>Silent trait </li></ul></ul><ul><ul><li>Not identifiable hematologically </li></ul></ul><ul><li>Deletion of 2  globulin gene </li></ul><ul><ul><li>Tha;assemia trait </li></ul></ul><ul><ul><li>Microcytic anemia  can be mistaken for iron deficiency anemia </li></ul></ul><ul><ul><li>Normal electrophoesis </li></ul></ul>
  31. 31.  Thalassemia <ul><li>Deletion of 3  globin genes </li></ul><ul><ul><li>Hb H disease </li></ul></ul><ul><ul><li>Marked microcytosis </li></ul></ul><ul><ul><li>Moderate anemia, mild splenomegaly, icteric, cholelithiasis </li></ul></ul><ul><ul><li>Transfusion is not usually necessary </li></ul></ul><ul><li>Deletion of 4  globin genes </li></ul><ul><ul><li>Profound anemia during fetal life </li></ul></ul><ul><ul><li>Hydrops ….. </li></ul></ul>
  32. 32. G6PD Deficiency <ul><li>Most important disease of the hexose monophosphate pathway </li></ul><ul><li>X-linked enxyme deficiency </li></ul><ul><li>Affcts more than 200 million people worldwide </li></ul><ul><li>G6PD is a central enzyme in the hexose monophosphate shunt of glucose metabolism </li></ul><ul><li>NADPH is produced  maintain glutathione in the redu…….tate </li></ul>
  33. 33. G6PD deficiency <ul><li>Gluthathione present in the RBC to neutralize agents that potentially oxidize or RBC membrane components </li></ul><ul><li>If reduced glutathione can’t be sustained to remove )2 radicals generated by oxidant  Hb precipitates (Heinz bodies)  RBC membrane is critically damage  hemolysis </li></ul>
  34. 34. G6PD deficiency <ul><li>Clinical Manifestattion: </li></ul><ul><ul><li>24-48 hr after oxidant drugs ingestion </li></ul></ul><ul><ul><li>Hemoglobinuria </li></ul></ul><ul><ul><li>Jaundice </li></ul></ul><ul><ul><li>Hb falls precipitously & life threatening </li></ul></ul>
  35. 35. G6PD deficiency <ul><li>Diagnosis </li></ul><ul><ul><li>History & PE </li></ul></ul><ul><ul><li>Lab </li></ul></ul><ul><ul><li>Reduced G6PD activity </li></ul></ul><ul><li>Treatment </li></ul><ul><ul><li>Prevention of hemolysis </li></ul></ul><ul><ul><li>Supportive </li></ul></ul>