Transcript Childhood Anemias

CHILDHOOD ANEMIA
TH
5 YEAR MEDICAL STUDENTS
Dr. Salma Al-Hadad
April 5th 2016
CHILDHOOD ANEMIA
Objectives:

To define anemia

To list the general causes of anemia

To list the signs and symptoms of anemia

To outline the steps for diagnosis of anemia

To describe the clinical presentation and management
of iron deficiency anemia in children

Define Hemolytic anemia & list the sites of hemolysis
CHILDHOOD ANEMIA
Definition of anemia
Anemia can be defined as a reduction in the hemoglobin
(Hb) concentration, hematocrit, or number of red blood
cells (RBC) per cubic milliliter below normal for age and
gender specific values
Anemia is a sign, not a disease
CHILDHOOD ANEMIA
Normal Hematopoiesis:
Hemoglobin is high at birth (14-20g/dl) and falls during
the initial 6 to 8 weeks of life to a physiologic nadir
(9.5g/dl) before it gradually increases to childhood levels
(10.5-14.5g/dl).

CHILDHOOD ANEMIA
Scenarios of presentation

A child who is feeling well but is found to have anemia
on a routine screening test,

A child who is “tired” and does not eat well,

A child who is being evaluated for other chronic
problems and is found to have anemia,

A child who is obviously pale, lethargic & requires
immediate assessment.
CHILDHOOD ANEMIA
General causes of anemia:
1. Poor production of red blood cells;

Nutritional deficiencies—iron, vitamin B12, folic acid.

Reduction in erythroid precursors— aplasia, BM
infiltration, leukemia.

Ineffective erythropoiesis: failure of apparently active
erythropoiesis to produce adequate number of
circulating red cells—Thalassaemia
2. Destruction of red blood cells i.e. hemolysis
3. Loss of red blood cells, i.e. bleeding
CHILDHOOD ANEMIA
A practical approach to the evaluation of the
anemic child

Step 1: confirm anemia by using age and gender specific
tables

Step 2: evaluate the red cell indices, of these; the mean
corpuscular volume (MCV) is the most useful. It enables the
classification of anemia by red blood cell size as microcytic,
normocytic, or macrocytic.

Step 3: assess WBC & platelet counts. Is the anemia isolated
or are other cell lines affected?

Step 4: Microscopic examination of the blood film can
assess the size, color, and shape of the red cells, also the
presence of abnormal cells.
CHILDHOOD ANEMIA
Symptoms:
Many children with anemia have no symptoms. That's why it's
important for children to have routine blood tests.
 Due to reduced supply of oxygen—Fatigue, dyspnea on exertion,
faintness, palpitations, dizziness, headache.
 In older subjects symptoms of cardiac failure, angina pectoris, or
confusion.
Other important parts of history:
o Patient History; Diet history (type/quantity of milk, Ingestion
of non-food items), medications, Activity level, Acute or
recent infection, chronic diseases.
o Family History; Ethnicity, Anemia, Jaundice, Splenomegaly,
Gallstones, Bleeding disorders, transfusions

CHILDHOOD ANEMIA
Adaptation to anemia
Reduction in delivery of oxygen to the tissues triggers a
variety of compensatory mechanisms, including
1.
Modulation of oxygen affinity—largely mediated by
an increase in red blood cell 2,3-biphosphoglycerate;
2.
Increased production of erythropoietin—the main
growth factor for red blood cell production;
3.
Redistribution of flow to benefit the myocardium,
brain, and muscle;
4.
Increase in cardiac output
CHILDHOOD ANEMIA
Clinical manifestations
The clinical picture depends on whether anemia is of
rapid or insidious onset.
1. Acute blood loss presents with features of
intravascular volume depletion
2. Anemia of gradual onset may (if mild) be
asymptomatic or simply manifest as slight fatigue and
pallor, or
3. (if more severe) present with features including
exertional dyspnea, tachycardia, palpitations, angina,
faintness, and signs of ‘cardiac failure’.
CHILDHOOD ANEMIA

Other signs related to the cause of anemia:
o
Jaundice points to a hemolytic process.
o
Splenomegaly may be seen in inherited hemolytic
anemia, malignancy, acute infection, or
hypersplenism secondary to portal hypertension.
o
Petechial rash indicates multiple cell lineages are
involved.
CHILDHOOD ANEMIA
Age-related differences in incidence
o
Newborn: Acute anemia most commonly occurs
among newborns.
o
Significant blood loss can occur from birth trauma or blood
exchange from the baby's mother (feto-maternal
transfusion) or the placenta.
o
Isoimmune anemia can result from maternal antibodies
crossing the placenta.
o
Abnormalities of fetal hemoglobin may cause anemia,
Deletion of α globin gene, unlike β globin gene mutation,
causes anemia in neonates.
CHILDHOOD ANEMIA
Age-related differences in incidence
o
o
o
Infancy: Nutritional anemia is common in infancy
because of the associated rapid growth
(necessitating an increase in red blood cell mass)
and dietary adjustments.
Early childhood: With exposure to new infections in
early childhood, the anemia of acute infection is
common.
Adolescence: is characterized by rapid growth and
vulnerability to nutritional anemia. In addition,
blood loss with heavy menstruation can be
observed in adolescent girls.
CASE 1




A 15-month-old male infant presented with poor appetite,
lethargy and progressive increasing paleness of body for last
2 months.
The child was born preterm at 32 weeks of gestation with a
birth weight of 1.8kg. He is predominantly on cow's milk
and consumes 500ml/day. No other significant medical
history. There is no history of passage of worms in stool.
There is no past history of blood transfusion or family
history of any disorder requiring repeated transfusions.
O/E; There is presence of conjunctival and palmar pallor but
no signs suggestive of cutaneous or mucosal bleeding.
Jugular venous pressure is not raised, and there is no
lymphadenopathy or organomegaly.
Hb 8g/dl, WBC 5,800/cmm, MCV 70 fL
CHILDHOOD ANEMIA
Nutritional Deficiency Anemia in Children
o
o
o
o
The term ‘nutritional anemia’ due to a
deficiency in one or several nutrients. The main
nutrients involved in the synthesis of
hemoglobin are iron, folic acid, and vitamin
B12.
In public health terms, iron deficiency is by far
the first cause of nutritional anemia worldwide.
Folic acid deficiency is less widespread and is
often observed with iron deficiency.
Vitamin B12 deficiency is far rarer.
IRON DEFICIENCY ANEMIA (IDA)
Causes of IDA:
1. Poor intake:

Inadequate dietary iron (unusual before 6 months but
becomes common at 9-24 month of age) by
consumption of large amounts of cow’s milk and of
foods not supplemented with iron.

Malnutrition
2. Increased demands:

low birth weight (low body iron stores then rapid
growth)
IRON DEFICIENCY ANEMIA (IDA)
Causes of IDA:
3. Blood loss:

A lesion of the GIT, such as a P.U., Meckel’s
diverticulum, a polyp, or by inflammatory BD.

Hookworm infestation is an important cause of iron
deficiency, e.g. Ancylosotma duodenale

Any site outside GIT, e.g. nose, trauma, lung, kidney
4. Malabsorption: e.g. celiac disease, partial gastrectomy
IRON DEFICIENCY ANEMIA (IDA)
Clinical features of IDA
1.
2.




3.
General symptoms and signs of anemia
Symptoms and signs specific to iron deficiency: Patients
with long-standing deficiency may develop changes like :
Nail flattening and koilonychia (concave nail),
Sore tongue and papillary atrophy,
Angular stomatitis (painful cracks appear at the angle of
the mouth).
Psychomotor delay and behavioral problems in young
children.
Symptoms and signs due to the underlying cause of iron
deficiency

Angular Stomatitis
Painful
cracks
appear
at the
angle
of the
mouth
IRON DEFICIENCY ANEMIA
What is A
Pale,
Smooth,
Burning
Tongue?
An alteration
in tongue
tissue
characteristic
of iron
deficiency.
Koilonychia:
"spoon nails” Iron deficiency anemia
IRON DEFICIENCY ANEMIA (IDA)
Diagnosis of IDA:
 The
blood counts:
 Low Hb
 Low MCV, MCH, and MCHC
 Blood film: microcytic hypochromic red cells
 Confirmatory tests:
 Low serum iron
 Increased total iron binding capacity
 Serum ferritin is probably the most useful of these
tests.
IRON DEFICIENCY ANEMIA (IDA)

MCV

MCH

MCHC

Fe

TIBC

TRANSFERIN SATURATION

FERRITIN
N
BLOOD FILMS
Normal red blood cells
Hypochromic Microcytic
IRON DEFICIENCY ANEMIA (IDA)
Treatment of IDA; Principles

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
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Use oral iron
Replace iron in total (8 wk. after blood values are
normal)
Establish and treat the cause (e.g. GI blood loss).
Therapeutic dose should be calculated in terms of
elemental iron
A daily total of 6 mg/kg of elemental iron in 3
divided doses
Response of IDA to iron is an important Dx. & Rx.
Feature.
IRON DEFICIENCY ANEMIA (IDA)
Response:

Reticulocyte count should rise in 5-10 days & Hb should increase
by 1 g/dl/wk.
Failure to respond to oral iron therapy due to:
1.
2.
3.
4.
5.
6.
7.
Wrong diagnosis (i.e. other cause of anemia like B-Thalassemia
minor)
Inadequate dose
Non-compliance (The child does not receive the prescribed
medication)
Malabsorption
Continued bleeding (as intestinal or pulmonary loss, or with
menstrual periods).
When iron is given in a form that is poorly absorbed
Severe infection
IRON DEFICIENCY ANEMIA (IDA)
Parenteral therapy; iron dextran
A parenteral iron preparation (iron dextran) is an
effective form of iron
Indications
 Poor compliance
 Intolerance of oral iron
 Severe bowel disease
 Chronic hemorrhage
 Acute diarrhea disorder
 The rise in Hb concentration is not faster than with
oral iron therapy.

IRON DEFICIENCY ANEMIA (IDA)
Prevention

Breast feeding until 5-6 mo, beyond 6 mo add Fe
supplementation

Iron-fortified formula for first 12 mo

Iron-fortified cereal early with solid foods

Avoid cow’s milk until 9-12 mo
CASE 2
B-THALASSEMIA MINOR

A 5-year-old male child presented with complaint of
pallor since early childhood.

no history of bleeding, weight loss, jaundice or blood
transfusion in past.

O/E active and playful. he was pale but there was no
icterus, cyanosis, clubbing, lymphadenopathy or
hepatosplenomegaly, the rest of the systemic
examination was normal.

Hb 9g/dl, MCV 60fL, hypochromic microcytic RBC.

RBC 6.5 million/cmm, Iron 35mg/dl, TIBC: 350 mg/dl,
Ferritin 25 ng/ml
Β-THALASSEMIA MINOR OR TRAIT
(HETEROZYGOUS Β0 OR Β+)
Clinical Features

Asymptomatic (physical examination is normal)
a. Discovered on routine blood test: slightly reduced
hemoglobin, basophilic stippling, low MCV, normal
RDW.

Discovered in family investigation or family history of
heterozygous or homozygous β-thalassemia.

Confirmed with hemoglobin electrophoresis,
demonstrating slightly decreased hemoglobin A (90–
95% typically), increased hemoglobin A2 (>4%);
hemoglobin F mildly elevated in 50% of cases.
CHILDHOOD ANEMIA
Hemolytic Anemia
Anemia, Reticulocytosis, Indirect hyperbilirubinemia

Describes a group of anemia that are characterized by
abnormal destruction of red cells. The hallmark of
these disorders is reduced life span of the red cells
rather than underproduction by the bone marrow.

The RBC normally survives about 120 days, but in
hemolysis the cell survival times are considerably
shortened.
PALLOR AND JAUNDICE
CHILDHOOD ANEMIA
Sites of hemolysis
I.
Intravascular hemolysis:
1.
2.
3.
4.
5.
6.
When RBC are rapidly destroyed within the
circulation, hemoglobin is liberated, e.g.
Glucose-6-phosphate dehydrogenase (G6PD) deficiency,
Hemolytic Uremic Syndrome
Disseminated intravascular coagulation(DIC)
Following transfusion of ABO incompatible blood,
Hemolytic anemia due to prosthetic cardiac valves and
Paroxysmal nocturnal hemoglobinuria (PNH)
CHILDHOOD ANEMIA
II.
Extravascular hemolysis:
The red cells are removed from the circulation by
macrophages in the RES, particularly the liver and spleen.
e.g. Autoimmune hemolytic anemia and Hereditary
spherocytosis.
III.
Intramedullary hemolysis:
Hemolysis may also be intramedullary when fragile RBC
precursors are destroyed in the bone marrow prior to
release into the circulation. e.g. Thalassemia major.
CHILDHOOD ANEMIA
Laboratory findings:
I. Increased RBC breakdown (intra or extra
vascular hemolysis)
Increased unconjugated serum bilirubin
2. Increased urine urobilinogen
3. Increased faecal stercobilinogen
4. Absent serum haptoglobin, because it becomes
saturated with Hb.
1.
II. Increased RBC production
1.
2.
Reticulocytosis
Bone marrow erythroid hyperplasia
CHILDHOOD ANEMIA
Laboratory findings
III. Damaged RBC
Morphology; microspherocytes, elliptocytes,
fragments
2. Osmotic fragility, autohemolysis
3. Shortened RBC survival
1.
40
41
42
43
CLASSIFICATION OF HEMOLYTIC ANEMIA
I. Inherited disorders
1.
RBC membrane defect; Hereditary Spherocytosis
and Hereditary Elliptocytosis
2.
Enzyme defect; Glucose –6 –phosphate
dehydrogenase and Pyruvate kinase deficiency
3.
Hemoglobin defects; Thalassemia syndromes and
Sickling disorders
CLASSIFICATION OF HEMOLYTIC ANEMIA
II. Acquired disorders

Immune mediated;
I. Auto immune (autoimmune hemolytic anemia),
II. Alloimmune (hemolytic disease of newborn,
hemolytic transfusion reaction)

Non-immune and trauma; valve prosthesis,
microangiopathy, infection, drugs or chemicals,
hypersplenism, secondary (liver and renal disease)
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