Hematologic Disorders

Anemia

By Wako.B
Pediatrics and Child Health Nursing
Bule Hora University
12/02/2023 1
 Introduction

Anemia

• Defined as a reduction in the hemoglobin concentration,

hematocrit, or number of red blood cells (RBC) per cubic
millimeter

• Results whenever the homeostatic balance between cell

Production(effective erythropoiesis) and loss is disrupted

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Comparison Of Hgb And Hct For Ages

Age (yrs ) Hemoglobin (g/dl) Hematocrit (%)

mean Lower limit mean Lower limit

0.5 - 1.9 12.5 11.0 37 33
2-4 12.5 11.0 38 34
5-7 13.0 11.5 39 35
8 - 11 13.5 12.0 40 36
12 – 14 female 13.5 12.0 41 36
12 – 14 male 14.0 12.5 43 37

15 – 17 female 14.0 12.0 41 36

15 – 17 male 15.0 13.0 46 38

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Physiologic classification of Anemia

• The major childhood anemia are classified according

to physiologic disturbance
A. Disorders of effective red cell production
B. Disorders of increased red cell destruction or loss

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 Disorders of effective red cell production

1. Marrow failure
a. Aplastic anemia
b. Marrow replacement

c. Pure red cell aplasia

2. Impaired erythropoietin production

• Chronic renal disease
• Chronic inflammation
• Protein malnutrition
• Hemoglobin mutants with decreased affinity for oxygen
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Disorders of effective red cell production

3. Abnormalities of cytoplasmic maturation

a. Iron deficiency

4. Abnormalities of nuclear maturation

a. Vitamin B12 deficiency
b. Folic acid deficiency

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Disorders of increased red cell destruction or loss

• Defects of hemoglobin
• a. Structural mutants (eg. HbSC) Hgb sickle cell disease

• Defects of the red cell membrane

• Defects of red cell metabolism

• Mechanical injury to the erythrocyte

• Acute/Chronic blood loss

• Hypersplenism

• Thermal injury to the erythrocyte

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 Evaluation of the anemic child
Step 1 : Determine the child is anemic using the Hb/Hct

Step 2: Evaluate the Red Cell Indices

• Enables classification of anemia

• microcytic
• normocytic or
• macrocytic.

Step 3. Asess the white blood cell (WBC) and platelet counts

Simple anemia Vs pancytopenia( decreased WBC, RBC& platelet

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 Transient Erythroblastopenia Of
Childhood(Tec)
• Is the most common acquired red cell aplasia occurring in children.

• Occurs in previously healthy child between 6 month and 3yrs of age

• The suppression of erythropoiesis has been linked to immunoglobulin (Ig)G,

IgM, and often follows a viral illness

• Virtually all children recover within 1-2 month.

• RBC transfusions may be necessary for severe anemia in the absence of signs

of early recovery

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 Anemia of Chronic Disease
• Also referred to as “anemia of inflammation and associated with
• Infection (e.g HIV, osteomyelitis) or
• Autoimmunity.
• rheumatoid arthritis
• systemic lupus erythematosus
• inflammatory bowel disease) as well as
• Some hematologic and solid malignancies

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Anemia of Chronic Disease…

• Erythrocytes have a mildly decreased life span

• Secondary to erythrophagocytosis by activated
macrophages
• Inflammation induces a blunted response and
relative resistance to EPO

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Anemia of Renal Disease…

• The anemia is usually normocytic, and the absolute

reticulocyte count is normal or low

• Causes:
• decreased EPO production
• absolute and/or functional iron deficiency due to chronic
blood loss and disturbances in the iron metabolic pathway
• Inflammation causes iron retention

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 TREATMENT
• Anemia of chronic disease does not respond to iron
unless there is concomitant deficiency.

• Transfusions raise the hemoglobin concentration

temporarily but are rarely indicated

• Recombinant human EPO

• Treatment with iron is usually necessary for an optimal

EPO effect.
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 Megaloblastic Anemias
• Is a macrocytic anemia characterized by ineffective
erythropoiesis,

• Active erythropoiesis associated with premature cell death and

decreased RBC output from the bone marrow.

• Usually an associated thrombocytopenia and leukopenia

• Childhood megaloblastic anemia result from folic acid or

vitamin B12 deficiency

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 Folic Acid Deficiency

• Folates are abundant in many foods, including green

vegetables, fruits, and animal organs (liver, kidney).

• Folic acid is absorbed throughout the small intestine,

and there is an active enterohepatic circulation.

• Body stores of folate are limited, and megaloblastic

anemia occurs after 2–3 months on a foliate-free diet

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 Etiology

1. Inadequate Folate Intake

2. Decreased Folate Absorption

• Malabsorption

3. Congenital abnormalities in Folate Metabolism

4. Drug-Induced Abnormalities in Folate Metabolism

• Pyrimethamine and
• Trimethoprim
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CLINICAL MANIFESTATIONS

• Irritability,

• Chronic diarrhea, and poor weight gain.

• Hemorrhages from thrombocytopenia

• Severe infections, failure to thrive,

• Neurologic abnormalities, and cognitive delays

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LABORATORY FINDINGS

• Variations in RBC shape and size are common

• Normal serum folic acid levels are 5-20 ng/mL; with

deficiency, levels are <3 ng/mL

• Serum activity of lactate dehydrogenase (LDH), a

marker of ineffective erythropoiesis, is markedly
elevated

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TREATMENT

• 0.5-1.0 mg/day of folic acid oral or parenteral should be

continued for 3-4 wks

• Maintenance therapy with a multivitamin (containing 0.2 mg

of folate) is adequate.

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Iron-Deficiency Anemia

• most widespread and common

• 30% of the global population

• newborn infant contains about 0.5 g of iron,

compared to 5 g of iron in adults.

• a dietary intake of 8-10 mg of iron daily is necessary

to maintain iron levels.

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ETIOLOGY

• Inadequate dietary iron usually occurs at 9-24 mo of age

• Blood loss

• Infections with hookworm, Trichuris trichiura,

Plasmodium, and Helicobacter pylori

• Adolescent growth spurt and menstrual blood loss

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CLINICAL MANIFESTATIONS
• Most children with iron deficiency are asymptomatic

• Pallor- not usually visible until the hgb falls to 7-8

g/dL

• When the hemoglobin level falls to <5 g/dL;

• irritability, anorexia, and lethargy develop, and systolic flow
murmurs are often heard.

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C/m…

• Iron deficiency has non hematologic systemic

effects
• impaired intellectual and motor functions
• Pica - the desire to ingest non-nutritive substances,
and
• Pagophagia - the desire to ingest ice

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 TREATMENT
• A daily total dose of 3-6 mg/kg of elemental iron in 3 divided
doses is adequate,

• Parenteral iron preparations are only used when malabsorption

is present

• Iron medication should be continued for 8 wk after blood

values normalize to reestablish iron stores.
• dietary counseling is usually necessary.
• Limiting bovine milk
• blood transfusion is rarely necessary

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 Summary

• Anemia is a sign of disease and not a final diagnosis

• The clinician’s goal is to define the underlying cause

• The anemia may be due to decreased production or

increased destruction or loss of red blood cells

• In our situation ,always consider the coexistence of

malnutrition and chronic bacterial infection or parasitic
disease like malaria, hookworm and schistosomiasis
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 Thank you

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