Anemia Anak 2
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Anemia Anak 2
dr. Bertha
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Physiologic Anemia of Infancy
• Normal newborn -> higher Hb & Ht, larger RBC
• Within 1st week progressive decline in Hb level begins and persists for 6-8 weeks Physiologic anemia
• Normaly reaches between 8-12 weeks old (Hb 9-11 g/dl)
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Physiologic Anemia of Infancy
• Cause– Decrease EPO production– Switch from fetal to adult Hb– Frequent blood sampling in preterm infant– Short RBC life span– Rapid growth
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Physiologic Anemia of Infancy
• Treatment– Ensuring that the diet contains essential
nutrients for hematoposis (folic acid & iron)– Transfusion– FPO– Complemental iron
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Facts!!
• Iron absorbed in proximal small intenstine mediated by duodenal protein (HFE, mobilfernin, hephaestin)
• Iron absorbed 2-3x more efficiently from human milk than cow’s milk
• Distribution of iron in the body:– Circulating RBC, muscle protein myoglobin
• 12% : Iron storage protein• …% :…..
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Facts!!
• Breast fed Infants should receive iron supplement from 4 month of age
• 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
• Anemia caused by un adequate duetary iron commoning 9-24 month of age
• Chronic iron deficiency anemia, causes:– Lession in GIT (peptic ulcer’s polyyp, Meckel
diverticulum, hemangioma, hookworm infestation)– Choronic diarhea
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Iron Deficieancy Anemia
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Stage of Iron Deficieancy Anemia
1. Depletion of iron store no functional changes
2. Iron store exhausted Tissue begin to have insufficient iron iron deficiency
Outright anemia isn’t yet detected, but this deficiency will impair kognitive, ↓physical capacity,↓ imunity
3. Iron deficiency anemia- Hb 7-9 g/dl : moderate anemia
- Hb < 7 g/dl : Severe anemia
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Clinical Manifestation
• Most importang sign : Pallor
• Iron deficiency Effect on neurologic and intelectual function (attention span, alertness, learning)
• When Hb <5 g/dl:– Irrittability, anorexia, tachycardia,cardiac
dilatation, systolic murmurs
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Progessive Iron deficiency
• 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) ↑
• RBC : microcytosis, hypochromia, poikilocytosis and increase RDW
• Reticulocyte normal or decrease• Thrombocytosis my occur (some struktural homology
between ertyhopoeitin and thombopoeitin). Few cases will have thrombocytopenia (in very severe iron deficiency anemia)
• Bone marrow hypercellular with erythroid hyperplasia
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Laboratory Finding
• Hb– Essential for diagnosis of anemia, easiets, less
expensive– Not very sensitive and specific for iron deficiency
(only the 3rd stage affects Hb synthesis)
• Ferritin– Currently considered the most important indicator– COncentrartion decrease even un the 1st stage of iron
deficiency– Most sensitive indicator– Influenced by nany factor : infection, inflamation↑
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Laboratory Findings
• Soluble Transferrin Receptor (sTfR)– Incresingly being used to determine iron
deficiency where infection is a factors– Not as sensitive as ferritin, but more sensitive
than Hb
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Differential Diagnosis
- and β- thalasemia trait
• Lead poisoning anemia
• Chronic inflamation of infection usually normocytic, but may be slightly microcytic. Serum iron level and iron binding capacity reduced, serum ferritin normal or elevated
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Treatment
• Oral administration of simple ferrous salts (sulfate, gluconate, fumarate)
• Therapeutic does: elemental iron 4-6 mg/kg/day on 3 divided dose
• Intolerance to oral iron theraphy is uncommon in young children. Older children and adolescent GI complaints
• Education re:diet• Parental iron preparation (iron dextran) is usually safe• Respone to parenteral iron is no more rapid or complate
than that obtained with proper oral administration of iron
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Treatment
• Medication should be continue for 8 week after blood value are normal
• Blood transfusion indocated only when the anemia is very severe
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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
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Anemia Hemolitic
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Definition
• Premature destruction of RBC
• If the rate of destruction exceeds the capacity of marrow to produce RBC Anemia
• Hemolytic process EPO released increase marrow activity reticulocytosis
• ↑ Hb degradation ↑ biliary excretion ↑ fecal urobilinogen
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Classification
• Intracellular– Membrane abnormality
• Hereditary spherocytosis, eliptocytosis, stomatocytosis
– Enzyme deficiency• G6DP deficiency
– Hemoglobinopathy• Thalasemia
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Clasification
• Extracellular– Antobodies/autoimune
• Autoimune hemolitic anemia
– Mechanical factor• Hypersplenism
– Plasma factor• Liver Disease• Infection
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Thalassemia
• Most common genetic disorder worldwide– Shorter RBC life span– Fetal Hb– RBC more sensitive to oxidatives stress
• More than 200 mutation• 3% of world population carry genes for β
thalasemia• In South East Asia 5-10% of the population
carry genes for thalassemia
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β Thalassemia
• β Thalassemia: excess -globin relative to β & globin chains
2β2 (HbA) 4 atau 22 (HbA2) atau 22 (HbF)
Thalassemia : fewer -globin chain2β2 (HbA) 4 (Bart’s Hb) atau 4
(HbH)
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• According to the degree of anemia:– Thalassemia trait– Minima– Minor– Intermedia– Major
• Genetic classification doesn’t define the phenotype
• Degree of anemia doesn’t predict the genetic clasification
β Thalassemia
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• Thalassemia intermedia (heterozygotes thalassemia)– Microcytic anemia– Hb about 7 g/dl– Extramedullary hematopoiesis
• Thalassemia minima & minor (heterozygotes thalassemia):– Phenotype more severe that trait, not as
severe……
β Thalassemia
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• Thalassemia trait:– Frequently misdiagnosis as iron deficiency– Short course iron therapy & evaluation to
separate patien who will need further evaluation
– Silent form of thalassemia
β Thalassemia
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Homozygous βo Thalassemia (Cooley’s anemia)
• Progessive hemolytic• Profound weakness & cardiac decompensation
after 6 month old if not treated• Thalassemic facies• Pathologic fracture• Hepatosplenomegaly• Cachexia• Expanded medullary space• Iron over
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Lab Finding
• Hb Electrophoresis
• Anemia
• Reticulocytosis
• Numerous nucleated RBC
• Microcytosis
• ↑ Unconjugated bilirubin
• ↑ Serum transferin
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Treatment
• Blood transfusion
• Iron chelation (desferoxamine)
• Splenectomy
• Bone marrow transplantation
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Thalassemia
• Most common is South East Asia
• ↑ Production of Bart’s Hb (4)
• Deletion mutation
• Need PCR for diagnosis
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Thalassemia
• Deletion of 1 globin gene– Silent trait– Not identifiable hematologically
• Deletion of 2 globulin gene– Tha;assemia trait– Microcytic anemia can be mistaken for iron
deficiency anemia– Normal electrophoesis
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Thalassemia
• Deletion of 3 globin genes– Hb H disease– Marked microcytosis– Moderate anemia, mild splenomegaly, icteric,
cholelithiasis– Transfusion is not usually necessary
• Deletion of 4 globin genes– Profound anemia during fetal life– Hydrops …..
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G6PD Deficiency
• Most important disease of the hexose monophosphate pathway
• X-linked enxyme deficiency• Affcts more than 200 million people worldwide• G6PD is a central enzyme in the hexose
monophosphate shunt of glucose metabolism• NADPH is produced maintain glutathione in
the redu…….tate
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G6PD deficiency
• Gluthathione present in the RBC to neutralize agents that potentially oxidize or RBC membrane components
• If reduced glutathione can’t be sustained to remove )2 radicals generated by oxidant Hb precipitates (Heinz bodies) RBC membrane is critically damage hemolysis
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G6PD deficiency
• Clinical Manifestattion:– 24-48 hr after oxidant drugs ingestion– Hemoglobinuria– Jaundice– Hb falls precipitously & life threatening
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G6PD deficiency
• Diagnosis– History & PE– Lab– Reduced G6PD activity
• Treatment– Prevention of hemolysis– Supportive