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
∀ α- 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 α thalassemia
 β Thalassemia
• β Thalassemia: excess α-globin relative to
β & γ globin chains
∀ α2β2 (HbA)  α4 atau α2δ2 (HbA2) atau
α2γ2 (HbF)

∀ α Thalassemia : fewer α-globin chain

∀ α2β2 (HbA)  γ4 (Bart’s Hb) atau δ4
(HbH)
 β Thalassemia
• 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
 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
 α Thalassemia
• Most common is South East Asia
• ↑ Production of Bart’s Hb (γ4)
• Deletion mutation
• Need PCR for diagnosis
 α Thalassemia
• Deletion of 1 α globin gene
– Silent trait
– Not identifiable hematologically
• Deletion of 2 α globulin gene
– Tha;assemia trait
– Microcytic anemia  can be mistaken for iron
deficiency anemia
– Normal electrophoesis
 α Thalassemia
• Deletion of 3 α globin genes
– Hb H disease
– Marked microcytosis
– Moderate anemia, mild splenomegaly, icteric,
cholelithiasis
– Transfusion is not usually necessary
• Deletion of 4 α 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