Childhood Anemia

Ghada Saad Abdelmotaleb

Assistant Professor of Pediatric

Objectives :

1-To know what is anemia.

2-To know different types of anemia.

3-How to diagnose and treat anemia?

Anemia

Definition :reduction of the red cell volume or HB concentration below the normal range for age.

1- Hemorrhagic Anemia.

2- Decrease RBCs production.

3-Hemolytic Anemia.

Classification of anemia

1-Hemorrhagic anemia.

2- RBCs production

RBCs precursors in BM

1- Pure red cell anemia:

congenital: Diamond-

Blackfan syndrome

acquired: eg.

Autoimmune, viral,

drugs,transient

erythroblastopenia of

childhood

2- Part of pancytopenia:

Aplastic panccytopenia:

-Congenital: Fanconi a.

-Acquired: 1ry (idiopathic)

2ry (eg. drugs,

Irradiation,

cytotoxic viral

inf, toxins)

Normal RBCs precursors in BM

Specific factor deficiency

(dyshemopoietic)

1-Chronic infection 2-Chronic inflammation 3-CRF

1- iron 2- B12 3- Folic A.

3- Hemolytic anemias:

Intracorpuscular causes Extracorpuscular causes

1- Cell wall defect:

Heridetary spherocytosis

Heridetary elliptocytosis

PNH

2-Enzyme defect:

G6PD

Pyruvate kinase

3- Hemoglobinopathies:

Thalassemias

Sickle cell anemia

1- Immune:

Isoimmune

Autoimmune

2- Non-immune:

Physical: eg. burn,

irradiation

Chemical: eg. Drugs, lead,

snake venom

Infection: eg. Malaria,

clostridium

Microangiopathies: eg.

DIC, HUS

Symptoms of anemia in children

Non-specific symptoms --Pallor. –Irritability . –Poor sleep quality. –Anorexia. –Poor concentration and school work. –Failure to thrive. •Dizziness / syncope. •Malaise, easy fatigue, impaired exercise .

tolerance -Palpitation. - Systolic murmur at base. -Cardiomegaly. - Congestive HF in

(sever cases).

• Anemia manifesting in neonatal period is

usually result of recent blood loss, iso-immunization,

congenital hemolytic anemia or congenital infection.

• Significant jaundice suggest congenital hemolytic anemia (e.g. Hereditary spherocytosis, G6PD, Pyruvate kinase deficiency).

• Nutritional iron deficiency is seldom responsible for anemia before 6 months of age in term infants (earlier in preterm infants).

Thalassemia syndrome more common

in South East Asians and Mediterranean.

Sickle cell disease more common in Africans.

Diet

Assess for dietary sources of iron, folic acid and

vitamin B12.

Pica suggest iron deficiency.

Laboratory investigations

CBC Haemoglobin : 8.8 g/dl Haemotocrite: 32% Red cell count:3.100000/cmm Red cell indices:MCV:68.0 flMCH:26.3 pgMCHC: 28.7% Leucocytic count: 6100 Basophils: 0%Esinophils: 2%Staff : 2%Segmented:53%Lymphocytes:41%Monocytes:2% Platelet count :210000 /cmm

The red cell indices in CBC

MCV (mean corpuscular volume)–The only red cell index directly measured by

the electronic counter.

–Reflects a quantitative defect in the production of Hb due to ↓hemoglobin synthesis.

– Classify anemia into microcytic, normocytic and macrocytic types

–Value must be interpreted with age.

•MCHC & MCH are calculate values & therefore

less accurate.

RDW (Red cell volume distribution width): –Reflects the variability in cell size and

measures the degree of anisocytosis. –Normal < 14.5%

–↑ ↑ in Fe deficiency anemia

–Normal in thalassemia trait

Physiological changes in red cell indices (Hb, Hct, RBC) with age

Very high level at birth–Relative hypoxemia in fetal life

• Physiological anemia at 2 –3 month of age–Dramatic reduction in erythropoiesis after birth

–Rapid growth in early infancy

• Gradual rise from childhood to adolescent

• Higher level in male vs female in adulthood

[Effect of androgens vs estrogen,

menstruation].

Reticulocyte count (RC) Reflects the rate at which new RBC are

produced;

Normally < 2% after 3 months; at birth up to 10%.

Reticulocytosis :in hemolysis and occur as response to treatment with iron after 48-72h from start == active BM

Anemia (Hb and Hct)

Retic or (N) RDW(N)

Iron deficiency anemia

(RDW)

Thalassemia trait

Lead poisoning

Chronic disease

Sidroblastic anemia

Thalassemia syndromes

(alpha and beta)

MCV

Further diagnostic test

MCV, RDW, Retic

Retic, RDW

Review of smear

Hb electrophoresis

Further diagnostic tests

Review of smear

To be continued

Microcytic anemia MCV, Retic or (N)

Serum iron

Ferritin

Serum iron

N or Ferritin

Serum iron

Ferritin

Suspect:

Iron deficiency

Suspect:

Thalassemia trait Lead poisoning Chronic disease (inflammation) Congenital sidroblastic anemia

Hb electrophoresis

Lead level

Bone marrow aspirate

(sidroblasts)

RDW() RDW(N)

Iron studies (Ferritin, S.iron)

Iron deficiency

Anemia of chronic disease

N Serum iron

N Ferritin

Thalassemia trait

Lead poisoning*

Congenital sidroblastic anemia

Iron therapeutic test (mild, well, non-sig hist, correlate with clinical data)

Retics

*Basophilic stippling in blood film

Causes of iron deficiency anaemia

1-Low birth weight and perinatal hemorrhage.

2-Inadequate dietary iron intake (cow milk , ↓supplementation).

3-Impaired absorption :chronic diarrhea, malabsorption, excessive tea, high phytate intake, antacids, low gastric acidity.

4-Chronic blood loss e.g. ankylostoma, cow milk allergy, peptic ulcer.

Microcytic anemia (DD)Get Iron panel: serum iron , TIBC, ferritin

Iron def. anemia

dec inc dec

Siderobla -stic anemia

inc dec inc

Thalasse mia -mia

inc/nl dec/nl inc/nl

Chronic disease

dec dec inc

Treatment

Prophylactic : 1-adequate iron to pregnant mother. 2- Breast feeding (exclusive). 3- Iron supplementation after 3rd month, earlier in

premature.

Curative : 1-ttt cause 2-Oral iron 6mg elemental/kg /day for 3-6m. 3-Parental iron therapy. 4-Packed RBCs in sever cases ---??HF.

Anemia (Hb and Hct)

Retic or (N) RDW(N)

Chronic disease Transient erythroblastopenia

of childhood Acute inflammation Acute hemorrhage Malignancy

Immune hemolysis RBCs membrane disorders

(HS, HE) RBCs enzyme defects

(G6PD, PK deficiency) Microangiopathic hemolysis

(HUS, DIC) Sickle cell anemia

MCV (N)

Further diagnostic tests

MCV, RDW, Retic

Retic, RDW

For other diseases (renal infection, , liver, metabolic)

Bone marrow biopsy

Further diagnostic tests

Osmotic fragility Enzyme assays

(G6PD, PK) Hb electrophoresis Coomb's test

Review of smear

Arranged according to clinical data

Anemia (Hb and Hct)

Retic or (N) RDW(N)

Folate deficiency B12 deficiency BM failure (aplastic anemia,

Fanconi anemia, DBA) Myelodysplastic syndrome Hypothyroidism Drug induced (anti-convulsants)

Active hemolysis with brisk

reticulocytosis

MCV

Further diagnostic test

MCV, RDW, Retic

Retic, RDW

Review of smear

Arranged according to clinical data

Bone marrow aspirate and biopsy

Folate, B12 level

Thyroid function test

Evaluate hemolysis

Folic acid deficiency anemia

Asynchrony between nuclear and cytoplasm maturation→ ↓DNA ,↑RNA → ↑ RBCs size.

↓ Serum folate , thrombocytopenia, hemorrahage

Vit. B12 deficiency (juvenile pernicious anemia) . {AR} - tongue smooth ,red, painful.

Neurological [sensory ataxia, parathesias , hyporeflexia, +ve Babiniski, clonus, coma]

Hemolytic anemias (general features)

Pallor (acute, chronic) + previous general features of anemia.

Tinge of jaundice {indirect hyper-bilirubinaemia}

Hepato-splenomegaly.

Stool may be dark. Urine (acute crisis →hemoglobinuria)

How to diagnosis?

Reticulocytosis >2% + nucleated RBCs in peripheral blood.

Hyper- bilirubinamia (indirect). High serum iron +low iron binding capacity. Low RBCs life span. low serum Haptoglobin. High urobilinogin. X-ray : skull, long and short bones, chest and

heart

Diagnosis of the cause

Shape of RBCs in blood smear.

Enzymatic assay.

Fragility test sickling preparation (Na metabisulfite) .

Hemoglobin electrophoresis.

Coombs test.

Alkaline denaturation test.

Hereditary Spherocytosis

AD, abnormal cell membrane. Due to abnormal in sub-membrane protein

skeleton (Spectrin) → ↑ permeability to Na →spherical RBCs + ↑ ATP use in Cation pump → premature aging, especially in spleen → destruction and chronic hemolysis.

C/P: Early onset (jaundice, neonatal anemia). Aplastic crisis (Human Parvo-virus). Gall bladder stone (pigment stone).

•Splenomegaly ??splenectomy Investigations :

All as previous + Blood smear Osmotic fragility test.

Treatment:

1- Splenectomy ??overwhelming sepsis [capsulated org.]

2- Blood transfusion

G.6.PD deficiency

XL- recessive (Glutathione) Oxidizing agents or infection. Acute hemolysis (abdominal pain, nausea, vomiting,

hemoglobinuria) . Anemia ,jaundice. Early neonatal presentation.

Diagnosis:Low Hb, high retic.Blood smear [Heinz inclusion bodies+ tear drop RBCs].Enzymatic activity assay(3m).

Treatment

Prophylactic :

avoid????????

Curative :

1-Mild : observation.

2-Sever: packed RBCS (10-20ml/kg)