Disorder of Blood in

Children

By- Rahul Dhaker

Asst. Professor
Ramsnehi College of Nursing
Bhilwara, Rajasthan- 311001
1
 Introduction
• Blood formation begins in the 4th week of
gestation.
• During the first 6 months of fetal life, liver
produce majority of the blood cells and at term
bone marrow carried out this function.
• Blood is composed of
– RBCs
– WBCs
– Platelets
– Plasma
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Cont… Introduction

• Blood disorders can affect any of the three main

components of blood:
– Red blood cells, which carry oxygen to the body's
tissues
– White blood cells, which fight infections
– Platelets, which help blood to clot

• Blood disorders can also affect the liquid portion

of blood, called plasma.
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Cont… Introduction

• Blood contains other important substances, such

as nutrients from food that has been processed by
the digestive system.

• Blood also carries hormones released by the

endocrine glands and carries them to the body
parts that need them.

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 Diagnostic procedure
• These are related to blood disorders should include
the followings-
– Blood examination
• CBC
• Hematocrit values
• PCV
• MCV
• MCHC
• MCH
• TLC
• DLC
• ESR
• Cell Morphology
• Hb etc.
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• MCHC- mean corpuscular hemoglobin
concentration
• MCH- mean corpuscular hemoglobin
– 27 to 33 picograms (pg) per cell ( Adult)

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Cont… Diagnostic procedure
• Bone marrow cytology
• Blood enzyme analysis
• Serum level
– Iron
– Ferritin
– Transferrin
– Lead
– Bilirubin
– Clotting factor
– Antibody titer etc.
• Hemoglobin electrophoresis
• Radiological investigation
• DNA Analysis
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 Anemia from inadequate RBC
production
• Iron deficiency anemia

• Lead poisoning

• Anemia due to chronic disease

• Anemia due to kidney disease

• Anemia from unusually rapid red blood cell

destruction
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Anemia resulting from increased RBC
destruction
• G6PD
• Hereditary spherocytosis
• Autoimmune hemolytic anemia
• Sickle cell disease

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 Diseases of the White Blood
Cells
• Neutropenia
• Human immunodeficiency virus (HIV)
• Leukemia
– AML
– CML
– ALL
– CLL

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 Diseases of Platelets
• Thrombocytopenia
• Immune thrombocytopenic purpura

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 Diseases of the Clotting System
• Hemophilia
• von Willebrand disease

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 Anemia
• Anemia is a common blood disorder that occurs
when the body has fewer red blood cells than
normal.
• Red blood cells carry oxygen throughout the body
using a protein called hemoglobin.
• If there aren’t enough of these cells or this protein,
anemia results.

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Cont… Anemia

• WHO proposed the cut off points of Hb level for

different age group for the diagnosis of anemia.

a) Children 6m to 6 yr- 11gm/dl

b) Children 6 yr to 14 yr 12 gm/dl

c) Above 14 yr Male 13 gm/dl

Female 12 gm/dl

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The cut-offs for haemaglobin and haemocrit

Haemoglobin
Population Group Haemocrit (%)
(g/dL)
Children 6 months to 5
years
11.0 33

Children 5-11 Years 11.5 34

Children 12-13 years 12.0 36
Non-pregnant women 12.0 36
Pregnant women 11.0 33
Men 13.0 39
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• Hemoglobin - the part of blood that distributes
oxygen from the lungs to tissues in the body.

• Hematocrit - the measurement of the percentage

of red blood cells found in a specific volume of
blood.

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 Cont… Anemia

• At all age the normal MCHC ( Mean corpuscular

hemoglobin concentration) should be 34.

• Value of MCHC below 34 indicate Hypochromic

RBC, which usually found in iron deficiency
anemia.

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• Pediatric anemia refers to a hemoglobin or hematocrit
level lower than the age-adjusted reference range for
healthy children.
• Physiologically, anemia is a condition in which
reduced hematocrit or hemoglobin levels lead to
diminished oxygen-carrying capacity that does not
optimally meet the metabolic demands of the body.
• Anemia is not a specific disease, but is a condition
caused by various underlying pathologic processes.
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• Anemia is often a symptom of a disease rather
than a disease itself. Anemia usually develops due
to the presence of one of the following:
– Excessive blood loss or hemorrhaging.

– Deficient production of red blood cells.

– Excessive red blood cell destruction.

– Both decreased production and excessive destruction

of red blood cells.

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 Grading of Anemia
• ICMR
• WHO
• Clinical presentation

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 Clinical grading Anemia
• According to clinical observation-
– Pallor observation in conjunctiva and mucous
membrane only- Mild Anemia

– Pallor observation in Skin – Moderate Anemia

– Pallor observation in palmar creases along with skin

and mucous membrane- Severe Anemia

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Clinical Manifestation
• Most symptoms of anemia are a result of the
decrease of oxygen in the cells or "hypoxia."
• However, each child may experience symptoms
differently.
– Abnormal paleness or lack of color of the skin.
– Increased heart rate (tachycardia).
– Breathlessness, or difficulty catching a breath
(dyspnea).
– fatigue
– Dizziness, or vertigo especially when standing.

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Cont… Clinical Manifestation
– Headache
– Irritability
– Sore or swollen tongue (glossitis)
– Jaundice, or yellowing of skin, eyes, and mouth
– Enlarged spleen or liver (splenomegaly,
hepatomegaly)
– Slow or delayed growth and development
– Impaired wound and tissue healing.

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 Complication
• Circulatory collapse
• Shock
• CCF
• Cardiac enlargement
• Systemic or local infection
• Growth retardation
• Mental retardation
• Sluggishness etc.
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Example

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 Type of Anemia
• Iron-deficiency anemia
• Megaloblastic anemia or macrocytic anemia-
• Hemolytic anemia
• Sickle cell anemia
• Thalassemia
• Chronic anemia
• Aplastic anemia
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Iron-deficiency anemia

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 Symptoms of Iron-deficiency anemia
• Iron-deficiency anemia develops over time.
• First, the amount of iron in the body goes down and
the child starts to have iron deficiency, where muscle
and brain function are affected.
– tiredness and weakness
– pale skin, especially around the hands, nails, and eyelids
– rapid heartbeat or a heart murmur
– irritability
– low appetite
– dizziness or feeling lightheaded
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How much iron do children need
Recommended amount
Age group
of iron a day
7 - 12 months 11 mg
1 - 3 years 7 mg
4 - 8 years 10 mg
9 - 13 years 8 mg
14 - 18 years, girls 15 mg
14 - 18 years, boys 11 mg
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Sickle cell Anemia

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• Sickle cell disease is an inherited blood disorder in
which red blood cells can become stiff, sticky and
often shaped like the letter C.
• Normal red blood cells are smooth and flexible,
like the letter O, and can move easily through
blood vessels.
•Sickle cells only live for
about 10 to 20 days, while
normal hemoglobin can
live up to 120 days.

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 Causes
• A genetic mutation is the cause of sickle cell anemia
• Tissue hypoxia. Tissue hypoxia and necrosis causes a
type of sickle cell crisis called the sickle crisis.
• Human parvovirus. Aplastic crisis results from
infection with the human parvovirus.
• Splenic infarction. Sequestration crisis results when
other organs pool the sickled cells, just like the
spleen.

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 Type

• Sickle cell anemia (HbSS)

• Sickle cell with hemoglobin C disease (HbSC)

• Hemoglobin S-β-thalassemia

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Pathophysiology

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 P/p
• Exposure. The sickle hemoglobin acquires a crystal-
like formation when exposed to low oxygen tension.
• Change in the shape. The oxygen level in venous
blood can be low enough to cause the erythrocyte to
lose its round, pliable, biconcave disk shape.
• Adherence. These long, rigid erythrocytes can adhere
to the endothelium of small vessels; when they adhere
to each other, blood flow to a region or organ is
reduced.
• Reversion. If the erythrocyte is again exposed to
adequate amounts of oxygen before the membrane
becomes too rigid, it can revert to normal shape.
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 Clinical Manifestation
• Anemia
• Infections, including pneumonia (lung infection) and
meningitis (infection of the lining of the brain). Signs
and symptoms may include:
– Fever
– Coughing
– Breathing problems
– Pain in the bones
– Headaches
• Pain Crisis
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Cont… Clinical manifestation

• Jaundice
• Leg ulcer
• Proteinuria ( Chronic stage)
• Delayed growth and development
• Delayed puberty
• Choleithiasis
• Cardiomegaly
• Avascular necrosis of the hip
• Bacterial sepsis or meningitis
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 Diagnostic Evaluation
• History collection :-
– Health history
– Noting Growth and development history
– Past hospitalization
– History of immunization including pneumococcal,
flu and meningococcal vaccination.
– Determine history of blood transfusion
– Current medication
– Present illness history
– History of hypoxia, illness and dehydration etc.
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Cont… Diagnostic Evaluation

• Physical examination:-
– Inspection and observation-
• Inspect the conjunctivae, palms, soles for pallor, skin pallor,
ulcers.
– Auscultation-
• Heart sound for murmur
– Palpation-
• Joint for warmth, tenderness and rang of motion (ROM),
• Swelling of hand or feet
• Palpate abdomen areas for tenderness.

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Cont… Diagnostic Evaluation

• Lab investigation
– Hb
– Platelet count
– ESR
– LFT
– Peripheral blood smear
– Blood cultures
– ABGs
– Pulmonary function tests
– Renal function (creatine, BUN, urinalysis)

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Cont… Diagnostic Evaluation

• Imaging studies
– X- Ray

– MRIs

– CT Scan

– Nuclear medicine scanning

– Abdominal Ultrasonography

– Echocardiography

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 Management
• The goals of treatment in SCD are symptom control and
management of disease complications.
• Treatment strategies include the following-
– Management of vaso-occlusive crisis (leads to sickle cell
crisis when the circulation of blood vessels is obstructed
by sickled red blood cells, causing ischemic injuries.)
– Management of chronic pain syndromes
– Management of chronic hemolytic anemia
– Prevention and treatment of infections
– Management of the complications and the various organ damage
syndromes associated with the disease
– Prevention of stroke
– Detection and treatment of pulmonary hypertension
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 Pharmacotherapy
• Antimetabolites
• Opioid analgesics (eg, aspirin, methadone, morphine
sulfate, acetaminophen, fentanyl, acetaminophen/codeine
etc.)
• Nonsteroidal analgesics
• Tricyclic antidepressants
• Antibiotics (eg, cefuroxime, amoxicillin/ clavulanate,
penicillin VK, ceftriaxone, azithromycin, cefaclor)
• Vaccines (eg, pneumococcal, meningococcal, influenza,
and recommended scheduled childhood/adult vaccinations)
• Vitamins (eg, folic acid)
• Antiemetics
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 Non-pharmacologic therapy
• Stem cell transplantation
• Transfusions
• Wound debridement (डिब्रीड्मन्ट)
• Physical therapy (physiotherapy)
• Heat and cold application
• Acupuncture and acupressure
• Transcutaneous electric nerve stimulation (TENS)

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 Complication
• Infection. Patients with sickle cell anemia are
unusually susceptible to infection, particularly
pneumonia and osteomyelitis.
• Stroke. Due to the decrease in oxygen supply because
of the sickling, stroke may occur.
• Renal failure. Blood flow is reduced to other body
tissues including the kidneys, which may lead to renal
failure.
• Heart failure. The heart compensates for the
decreased blood distribution by pumping more blood,
and it may ultimately fail if it wears out.
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 Idiopathic
Thrombocytopenic
Purpura (ITP)

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 Introduction
• Idiopathic thrombocytopenic purpura is a blood
disorder characterized by an abnormal decrease in
the number of platelets in the blood.

• Platelets are cells in the blood that help stop

bleeding. A decrease in platelets can result in easy
bruising, bleeding gums and internal bleeding.

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 Cont… Introduction

• ITP is also called immune thrombocytopenic purpura

or idiopathic thrombocytopenic purpura or
autoimmune thrombocytopenic purpura or primary
immune thrombocytopenic purpura.
• "Idiopathic" means the cause is unknown.
• "Thrombocytopenia" means a decreased number of
platelets in the blood.
• "Purpura" refers to the purple discoloring of the skin,
as with a bruise.(Definition of purpura - a rash of purple spots on the
skin caused by internal bleeding from small blood vessels.)
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 Etiology
• The cause of ITP is unknown
• Increased breakdown of platelets.
• Viral infection

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 Type
• Acute thrombocytopenic purpura (पर्प्यरु )is most commonly
seen in young children (2 to 6 years old). The symptoms
may follow a viral illness, such as chicken pox. Acute ITP
usually has a very sudden onset, and the symptoms usually
disappear in less than six months (often within a few
weeks). The disorder usually does not recur. Acute ITP is
the most common form of the disorder.

• Chronic thrombocytopenic purpura can happen at any

age, and the symptoms can last a minimum of six months or
several years. It is more common in adults than in children,
but it does affect adolescents. Two to three times more
females have ITP than males. Chronic ITP can recur often
and requires continual follow-up care with a hematologist.
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Clinical Manifestations
• Petechiae. Petechiae appear when
capillaries bleed, leaking blood into the skin.

• Purpura- Easy or excessive bruising

or purpura is dominant; if bone marrow
megakaryocytes cannot increase production
and maintain a normal number of circulating
platelets, thrombocytopenia and purpura
develop.
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• Hemorrhage. Hemorrhagic
bullae on mucous membranes, gingival
bleeding, signs of GI bleeding, retinal
hemorrhages, and evidence of
intracranial hemorrhage, with possible
neurologic symptoms; the bleeding
results from unusually low levels of
platelets — the cells that help blood
clot

• Nosebleeds
• Bleeding in the mouth
and/or in and around the
gums
• Blood in the vomit, urine or
stool

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• CNS Hemorrhage
• Severe manifestation
– Hematuria
– GI bleeding
– Severe nose bleed
– Menorrhagia
– Intracranial hemorrhage etc.

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 Diagnostic Evaluation
• History taking
– Drug therapy
– X-ray exposure
– Use of spray
– Exposure to toxin substance
– Recent illness
– Any immune system problem
• Physical examination
• CBC- platelet count , BT, CT, other findings
• Bone marrow aspiration or biopsy
• Blood smear
• Platelet antibody
• Urine test
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 Management
• Specific treatment for ITP will be determined by
your child's physician based on:
– Child's age, overall health and medical history
– Type of disorder (acute versus chronic)
– Severity and extent of the disorder
– Child's tolerance for specific medications, procedures
or therapies
– Expectations for the course of the disease
– Parent opinion or preference

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 Cont…Management
• Supportive management to be provided with blood
and platelet transfusion to prevent and treatment
bleeding episodes.
• Steroids, which help prevent bleeding by
decreasing the rate of platelet destruction.
Steroids, if effective, will result in an increase in
platelet counts seen within two to three weeks.
Side effects may include irritability, stomach
irritation, weight gain, hypertension and acne.
– Usual dose 1 to 2 mg/kg/day of predinosolone for 2 to
3 weeks followed by tapering dose over next 1 to 2
weeks.
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 Cont…Management
• Intravenous gamma globulin (IV- IgG)- is
administered with 2gm/kg of total dose to protect
platelets antibody.
• Anti Rh immune globulin therapy-
• Infection treatment
• Splenectomy -
• Hormone therapy — Teenage girls may need to
take hormones to stop their menstrual cycle when
their platelets are low if excessive bleeding occurs.

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 Cont…Management
• Lifestyle and home remedies-
– Avoid activities that could cause injury. Ask your doctor
which activities are safe for you. Contact sports — such as
boxing, martial arts and football — carry a high risk of
injury.
– Drink alcohol in moderation, if at all. Alcohol slows the
production of platelets in your body. Ask your doctor
whether it's OK for you to drink alcohol.
– Watch for signs of infection. If you've had your spleen
removed, be alert for any signs of infection, including fever,
and seek prompt treatment. Infection in someone who has
had a splenectomy may be more severe than in someone
who still has a spleen.
– Use caution with over-the-counter
medications. Nonprescription drugs such as aspirin and
ibuprofen (Advil, Motrin IB, others) can impair platelet
function.
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