Anemia Anak 2

dr. Bertha

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)

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

Physiologic Anemia of Infancy

• Treatment– Ensuring that the diet contains essential

nutrients for hematoposis (folic acid & iron)– Transfusion– FPO– Complemental iron

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• …% :…..

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

Iron Deficieancy Anemia

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

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

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

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↑

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

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

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

Treatment

• Medication should be continue for 8 week after blood value are normal

• Blood transfusion indocated only when the anemia is very severe

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

Anemia Hemolitic

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

Classification

• Intracellular– Membrane abnormality

• Hereditary spherocytosis, eliptocytosis, stomatocytosis

– Enzyme deficiency• G6DP deficiency

– Hemoglobinopathy• Thalasemia

Clasification

• Extracellular– Antobodies/autoimune

• Autoimune hemolitic anemia

– Mechanical factor• Hypersplenism

– Plasma factor• Liver Disease• Infection

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

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)

• 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

• 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

• 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

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

Lab Finding

• Hb Electrophoresis

• Anemia

• Reticulocytosis

• Numerous nucleated RBC

• Microcytosis

• ↑ Unconjugated bilirubin

• ↑ Serum transferin

Treatment

• Blood transfusion

• Iron chelation (desferoxamine)

• Splenectomy

• Bone marrow transplantation

Thalassemia

• Most common is South East Asia

• ↑ Production of Bart’s Hb (4)

• Deletion mutation

• Need PCR for diagnosis

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

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 …..

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

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

G6PD deficiency

• Clinical Manifestattion:– 24-48 hr after oxidant drugs ingestion– Hemoglobinuria– Jaundice– Hb falls precipitously & life threatening

G6PD deficiency

• Diagnosis– History & PE– Lab– Reduced G6PD activity

• Treatment– Prevention of hemolysis– Supportive