Anemia in Infancy

Dr. Sachitra Rathod

M.D. Pediatrics, Fellow in Allergy and Clinical
Immunology
Assistant Professor, Pediatrics
ESIC Medical College and Hospital
 Definition

Reduction in Red
Reduction in Red Cell
cell VOLUME
MASS
 Definition
• Haemoglobin (Hb) − This is a measure of the
concentration of the RBC pigment haemoglobin in
whole blood, expressed as grams per 100 mL (dL)
of whole blood.
 • Blood being
Blood loss produced but lost
from body.

Inadequate • Blood is not being

production produced at all.

• Blood is being
produced in
Destruction
of Blood cells marrow but getting
destroyed in
circulation
 Etiology
Birth to Three months Three months to Six months
• Physiologic Anemia • Hemoglobinopathy

• Pathologic anemia: • Nutritional deficiency

o blood loss,
o immune hemolytic disease,
congenital infection
o congenital hemolytic anemia
 Etiology- Blood Loss
• Antenatal

• Intranatal and

• Postnatal
 Diagnosis and Management
• History of blood loss.

• Pallor, tachycardia and shock.

• Mx: stabilizing the patient (ABC), PRBC

transfusion
Physiologic anemia of Infancy
 Physiologic anemia of Infancy
• It is physiologic adaptation to extra uterine life

• Lowest level reached by 8 to 12 weeks.

• Level is usually not below 10 mg/dl

• No treatment is needed.
 Anemia of Prematurity
• Premature babies:
o Nadir by 3-6 weeks and is more severe

• AOP:
• EPO with iron/Transfusions/Iron therapy: 1-
2mg/kg/day from one month to one year of age.
 Physiologic/pathologic??
• Anaemia (HGB <13.5 g/dL) within the first month of
life

• Anaemia with lower Hb level than is typically seen

with physiologic anemia (<9 g/dL)

• Signs of hemolysis (jaundice or dark urine) or

symptoms of anaemia (e.g. irritability or poor
feeding)
Etiology: Inadequate production
 Pure Red cell Aplasia
• Usually caused by parvo virus infection

• Cytotoxic to marrow erythroid precursors.

• Characteristic nuclear inclusions in erythroblasts and

giant pronormoblasts under light microscopy in bone
marrow.

• Infection is usually transient with recovery in two weeks.

• In HS, autoimmune hemolytic anemia or sickle cell
disease, Parvovirus B 19 infection can cause aplastic
crisis.

• Dx: IgG and IgM titres and PCR for the virus.

• PRBC Tx : aplastic crises or when associated with severe

symptoms.

• If infection occurs in utero, can result in hydrops fetalis

and anemia.
 Congenital Hypoplastic Anemia
• DBA: bone marrow failure syndrome.
• Diagnosed in first year of life.
• Anemia+ reticulocytopenia
• Profound anemia by 2-3 months of age.
• 40-50% have congenital anomalies: craniofacial
and skeletal.
• Diagnosis: Macrocytic anemia, low retic count,
elevated HbF,
• Elevated adenosine deaminase activity.
• Differential Diagnosis: TEC, Pure Red cell aplasia.

• Treatment: Blood transfusions till 1 year of age,

corticosteroids, HSCT.
• Target Hb should be >9 g/dl.
Transient Erythroblastopenia of Childhood

• Most common acquired red cell aplasia

• Severe transient hypoplastic anemia, 6mo-3 yrs

• Often follows viral illness.

• Reticulocytopenia, NC anemia

• RBC adenosine deaminase levels are normal.

• Recover within 1-2 mo.

• PRBC Tx only in severe anemia.

• Any child with TEC requiring more than one

blood transfusion should be evaluated for other
causes.
Etiology: Nutritional anemias
Etiology: Hemolytic Anemia

Immune Non –Immune

mediated mediated
 Hemolytic Anemias
• Hereditary Spherocytosis
• Hereditary Elliptocytosis
• Pyruvate Kinase deficiency
• G6PD Deficiency
 Hereditary Spherocytosis

• Autosomal dominant disorder

• Neonatal period: hemolysis
(anemia/hyperbilirubinemia)
• Usually need blood transfusions, phototherapy
or exchange transfusion.
• Dx: Anemia, Low MCV, High MCHC with Low
RDW, Speherocytes in P.S.
 Hereditary Elliptocytosis
• Elliptical deformation of cell over time.

• Severity varies: No symptomatology to severe anemia.

• Usually an incidental finding on p.smear.

• Dx: P.smear and signs of hemolysis

• Rx: No treatment to needing transfusions, splenectomy.

 Hemoglobinopathies
• Thalassemia
• Sickle cell anemia
 Hemoglobin
• Chr 16 codes for alpha genes.
• CHr 11 codes for beta gene.
• Fetal Hb: ⍺2𝛾2
• HbA: ⍺2β2
• HbA2: ⍺2δ2
 Sickle Cell Anemia
• Mutation on β chain at 6th position resulting in
valine instead of glutamine.

• Conformational change in RBC and produces

sickling in deoxygenated state.
 Clinical features
• Anemia
• Jaundice
• Neutrophilia, thrombocytosis
• Splenomegaly in infancy
• P.smear: Sickle cells, HJ bodies,
• Hb Electrophoresis shows HbSS pattern.
 Splenic Sequestration
• Seen between 6 to 24 months.
• Blood gets pooled up in spleen due to sickling of
cells
• Hypovolemia and shock.
• Sudden drop in Hb.
• Transfuse PRBC immediately.
 Mangement
• Pain relief
• Hydration
• Supplemental oxygen
• Empiric antibiotics
• Blood transfusion
• Hydroxyurea: >9 months
 Thalassemia
• “Thalassemia refers to a group of genetic disorders
of globin-chain production in which there is an
imbalance between the α-globin and β-globin chain
production.”

• The primary pathology in the thalassemia syndromes

stems from the quantity of globin produced, whereas
the primary pathology in sickle cell disease is related
to the quality of β-globin produced.”
 Thalassemia
 𝜷 𝒕𝒉𝒂𝒍𝒂𝒔𝒔𝒆𝒎𝒊𝒂 ⍺ thalassemia
• No beta globin chain • No ⍺ globin chain: ⍺-
production-β0 • ⍺ globin chain reduced: ⍺+
• Beta globin chain reduced β+
 Clinical Features
• Anemia, HSM
• Abnormal facies
• Indirect hyper-bilirubinemia
• Failure to thrive
• Growth retardation
• Organ dysfunction
 Investigations
• CBP: Microcytic hypochromic, reticulocytosis

• Leucopenia and thrombocytopenia

• HbF and HbA2 increased

• S. Ferritin is increased.
 Management

Chelation Supportive
Transfusion
care

Splenectomy
 Transfusion
• Transfusion regime:

 Base line Hb of 9.5 to 10.5g/dL

 Allows for normal growth
 Transfusion is needed every 3-4 weeks
 Watch for iron overload
 Chelation
• Desferrioxamine, Deferasirox and Deferiprone is
used for chelation.

• Desferrioxamine is used s.c or i.v.

• Done when patient is significantly iron loaded.

 Splenectomy
• Declining now due to regular transfusion.

• Advised in transfusion dependent patients with

hypersplenism.

• Ensure immunization against pneumococcus, HiB

before splenectomy.
 References
• Excerpt From: Kliegman, Robert M. “Nelson
Textbook of Pediatrics E-Book”.

• Manual of Pediatric Hematology and Oncology,

Philip Lanzkowsky, 4th Edition.
Thank you