Diroders of hematologial system

DISORDERS OF
HEMATOLOGICAL
SYSTEM
BY-
RAMYA DEEPTHI P
ASST PROFESSOR
VIJAY MARIE COLLGE OF NURSING

DISORDERS OF BLOOD
A. ANEMIA
B. THALASSEMIA
C. HEMOPHILIA
D. LEUKEMIA
E. PURPURA

Definition
Anemia is defined as reduction in the
volume of red blood cells or in-concentration
of hemoglobin, below the lower limit of the
normal range for age and sex of the
individual

CLASSIFICATION
Two widely accepted classification of
anemia are-
I. Classification based on Morphology
II. Classification based on Etiology

BASED ON MORPHOLOGY
Based on mean corpuscular volume, anemia is of three types-
1. Microcytic : abnormally small RBC’s are present in iron deficiency
anemia and certain non iron deficiency anemia's like sideroblastic
anemia and thalassemia.
2. Normocytic anemia : RBC’s are normal in shape but anemia
occurs due to blood loss, Hemolysis or bone marrow failure.
3. Macrocytic anemia : in this type, the RBC’s are abnormally large in
shape. It is usually due to vit-B12 or folic acid deficiency.

2. Anemia due to impaired red cell
production
a. Iron deficiency anemia
b. Vit B12 and folic acid deficiency
anemia
c. Aplastic anemia
d. Aplasia of pure red blood cells
e. Anemia due to infection
f. Anemia in renal disease
g. Anemia in liver disease
h. Anemia in disseminated malignancy
i. Anemia in endocrinopathies
j. Leukemia
k. Myelosclerosis
l. Multiple myeloma
m. Sickle cell anemia
1. Anemia due to blood loss
a. Acute post hemorrhagic anemia
b. Chronic post hemorrhagic anemia
2. Anemia due to increases destruction of red
blood cells
A. Anemia due to intracorpuscular defect
a) Sickle cell anemia
b) Thalassemia
B. Anemia due to extracorpuscular defect
a) Hemolytic disease of new born
b) Effect of toxic drugs
c) Effect of venoms or poisoning from
substances like lead
d) Thermal injury or burns
e) Transfusion reactions
f) Infections like infectious mononucleosis
BASED ON ETIOLOGY

Diagnosis of anemia
Anemia can be diagnosed based on the basis of –
1. Hemoglobin estimation
2. Peripheral blood film examination
I. Variation in size of RBC’s
II. Variation in shape of RBC’s
III. Inadequate hemoglobin content
3. Red cell indices
4. Leucocyte, reticulocyte and platelet count
5. Bone marrow aspiration

I Hemoglobin estimation
If the hemoglobin amount is less than the
expected normal range and sex then the
person is said to be anemic.

II Peripheral blood film
examination
Examination of a well prepared and stained peripheral blood smear is most
helpful in diagnosis of anemia. The following abnormalities can be seen in
the blood smear-
i. Variation in size of RBC’s: normally the diameter of RBC’s is 6.7 – 7.7
um. Increased variation in size of the RBC’s is termed as Variation in
shape of ‘Anisocytosis’
ii. Variation in shape of RBC’s: increased variation in shape of RBC’s is
known as ‘Poikilocytosis’. It is seen in megaloblastic anemia,
thalassemia, iron deficiency anemia.
iii. Inadequate hemoglobin content: normally on staining red cells, they
appear pink in color. The intensity of pink color depends on the level of HB
present. A low Hb Content may be low in iron content known as
‘Hypochromasia’ and increases content known as Hyperchromasia

III Red cell indices
Measurement of red cell indices help to diagnose the type
and severity of anemia. In iron deficiency anemia MCV,
MCH, MCHC are reduced. In megaloblastic anemia
MCV is more than the normal range.
MCV- mean corpuscular volume
MCHC mean corpuscular hemoglobin concentration
MCH- mean corpuscular hemoglobin

IV Leucocytes, Reticulocyte and
platelet count
1. Estimation of leucocyte and PC helps in
distinguishing pure anemia from
pancytopenia, in which red cells,
granulocytes and platelets all are
recorded.
2. In anemia due to hemolysis or blood loss,
neutrophil and PC are elevated.
3. In infection, leukemia, leucocytes counts
are elevated and immatutre leucocytes
appears in blood.
4. After acute hemorrhage or hemolysis,
reticulocyte count rises with in 2-3 days.
5. An impaired reticulocyte count indicates
impaired bone marrow function.

Bone marrow examination
Bone marrow aspiration may be performed
because cellular changes with in marrow are
diagnostic of many hematological conditions
like leukemia.

Introduction
• Iron deficiency anemia is the most
common hematological disorder of infancy
and childhood.
• It is caused by lack of iron for the
synthesis of hemoglobin

Iron absorption and metabolism
• Iron required for Hb synthesis is
derived from two sources- ingestion of
food rich in iron, recycling of iron form
broken RBC’s.
• Dietary form is absorbed in the small
intestine and either passed into blood
stream or stored in intestinal epithelial
cells as ferritin.
• The iron in blood stream binds to iron-
transport molecule- transferritin
• Then it is delivered to RBC’s in bone
marrow
• It combines with other components of
hemoglobin.

ETIOLOGY
SEVERAL FACTORS MAY CONTRIBUTE TO IRON
DEFICIENCY ANEMIA INCLUDINH-
1. Increased blood loss: peptic ulcer, polyps,
hematuria, parasitic infestations and epistaxis.
2. Insufficient iron supply at birth: infants born to
anemic mothers receive inadequate iron form mother
during IU life. Also, if baby is preterm or had lost
blood before or during process of birth, he is prone to
iron deficiency anemia.

Contd…
3. Insufficient iron intake: maternal supply of iron is
sufficient for first 4-5 months of infants life. Maternal
insufficiency of iron to baby will lead to development
of anemia with in 2 months of life. Infant fed on cow’s
milk should be given iron supplementation as it is
poor in iron.
4. Impaired iron absorption: mal absorption syndrome,
chronic diarrhea, intake of antacids and tea after
meals.

Pathophysiology
• Due to any etiological factor, when sufficient iron is not available for
hemoglobin synthesis, the production of hemoglobin is decreased.
• As the hemoglobin decreases, the newly formed RBC’s become
smaller (Microcytic) and less filled with hemoglobin (Hypochromic)
• This results in decreased Hb levels and reduced oxygen carrying
capacity of blood

Contd..
Development of anemia
progresses in 3 stages.
1. Firstly, iron stores are
depleted in an attempt to
supply iron for erythropoiesis.
2. In the second step, iron
supply for erythropoiesis is
reduced without development
of anemia.
3. The final step is development
of frank anemia with
Microcytic and Hypochromic
RBC’s

CLINICAL FEATURES
• The most clinical feature of anemia is PALLOR.
• When Hb levels falls 5-6 gm/dl, following features are developed in child-
i. Irritability
ii. Constipation
iii. Cardiac enlargement
iv. Tachycardia
v. weakness
vi. Dyspnea
vii. Poor attention
viii. Reduced alertness

Diagnostic evaluation
Anemia may be mild, moderate, severe. It can be diagnosed on
the following basis-
I. History of child
II. Blood test
III. Peripheral blood smear
IV. Stool test

i. History of the child
• a through dietary history is essential in making diagnosis of iron
deficiency of anemia.
• The history usually reveals high milk intake and low intake of
iron containing foods.

ii. Blood test
• Hemoglobin level is below 11gm/dl
• Hematocrit is below 33%
• MCV is bellow 70um3 in infants and below 75 um3 in
children
• Reticulocyte count is reduced
• Serum ferritin concentration is below 10 mg/dl
• Serum iron value is below 30 ug/dl
• Total iron binding capacity is elevated to 350 um/ dl in an
attempt to absorb more iron

iii. Peripheral blood smear
• The smear shows microcytric and hypochromic red cells which
may vary in shape (poikilocytosis) and size (anisocytosis).

iv. Stool test
• Stool is tested for presence of occult blood which indicates
bleeding form gastrointestinal tract.

MANAGEMENT
I. Oral iron therapy
II. Parenteral iron therapy
III. Blood transfusion

I. ORAL IRON THERAPY
• A therapeutic dose of 6mg/kg/day of elemental iron given orally
in 3 divided doses provide an optional amount of iron needed
for hemoglobin synthesis.
• With this dose, the hemoglobin level should rise by 0.4gm/dl
per day.
• Oral iron therapy should be continued for at least 6-8 weeks
after the hemoglobin has reached normal level.

II. PARENTERAL IRON
THERAPY
• A parenteral iron preparation, iron dextran which contains 50mg
elemental iron per ml is used, when therapeutic result of oral iron
therapy is not achieved.
• Iron requirement of the body is determined by the following equation-
Iron requirement (mg)=wt (kg) X Hb deficit (g/dl) X 4
• Daily dose of iron dextran should be limited to 50 mg in infants and
100 mg in adults.
• Ity is administered intramuscularly using Z-tract method, deeply into
large muscle mass.
• Side effects of parenteral iron include pain, chills, fever, arthralgia,
shock and even fatal anaphylaxis.

III. BLOOD TRANSFUSION
• When anemia is severe , the
child may go into congestive
heart failure.
• When the hemoglobin levels
is below 4gm/dl, only packed
cells should be given slowly.
• Also one or two doses of
frusemide 1-2mg/kg,
intravenously should be given
to prevent circulatory
overload.

NURSING MANAGEMENT
• Nursing management focuses on parental education, It consists
of Teaching about -
1. proper administration of iron supplements
2. Side effects of iron therapy
3. Improving dietary iron intake

i. proper administration of iron
supplements
• Iron medication should be given between meals
• Medication should not be administered with milk or tea which
reduces its absorption
• It should be given with any form of ascorbic acid such as citrus
fruit or juice which aids in absorption
• Liquid iron preparation can stain teeth temporarily so it should
be given to infants with medicine dropper or syringe placed
towards the back of the mouth
• Older teeth can take this solution through straw followed by
rinsing mouth or brushing

ii. Side effects of iron therapy
• Abdominal cramps
• Nausea
• Vomiting
• Diarrhoea or constipation
• Should be given with meals to prevent
gastrointestinal irritation

iii. Improving dietary iron intake
• Iron rich foods-
 Meat
 Liver
 Kidney
 Egg yolk
 Green leafy vegetables
 Fruits like apple
 At the time of weaning iron rich diet should be given to infant
 Food should be prepared in utensils made up of iron, to
increase the iron content of food.

iv. prevention
• Imp responsibility is to educate parents on prevention.
 Iron fortified milk formulas should be used for non-breast fed
infants.
 Prevent worm infestations
 Antihelmenthic drugs to manage infestations
 Cook food in iron utensils.
 Iron supplements should be administered to preterm and low
birth weight infants having low iron stores.

INTRODUCTION
• When deficiency of vitamin B12 or folic acid occurs,
the rate of DNA and RNA synthesis is reduced,
delaying cell division. The cells thus get extra time
between divisions so they grow larger than normal
resulting in formation of Megaloblasts.
• Deficiency of vitamin B12 and folic acid impairs the
maturation of erythrocytes leading to formation of
abnormally large erythrocytes known as Megaloblasts.

ETIOLOGY
• Results from deficiency of vitamin B12
and folic acid which may occur in
following conditions-
 Inadequate dietary intake of vit B12
and Folic acid
 Mal-absorption
 Treatment with anticonvulsants
 Chemotherapy
 Excess urinary folate loss ( active liver
disease and congestive heart failure)
 Intrinsic defect of folic acid absorption

Clinical features
• Pallor
• Sick look
• Irritability
• Anorexia
• Failure to thrive
• Increased pigmentation on back of hands, fingers and
nose
• Glossitis
• Tremors and developmental retrogression (rare)
• Neurological manifestations like numbness, parasthesia,
weakness, ataxia and diminished reflexes.

Diagnosis
• Peripheral blood smear
• Serum vitamin B12 and folic acid assay

Management
• Acute deficiency of vitamin B12 and folic acid can be
managed by-
 Administration of folic acid in dosage of 2-5mg/day
 Vitamin B12 administration of 1ug/ day.

Definition
• Aplastic anemia occurs due to marked reduction in precursor
stem cells resulting in production of inadequate number of
erythrocytes, leucocytes and platelets.

Incidence and Etiology
• It can be either hereditary or acquired.
• The hereditary form of Aplastic anemia is known as “Fanconi’s anemia”.
• It is rare autosomal recessive disorder.
• Acquired Aplastic anemia can be either idiopathic or may result from
secondary causes.
• The most common cause of Aplastic anemia is autoimmune suppression of
blood cell production.
• Certain toxins and pharmacology agents implicated in the development of
Aplastic anemia are-
1. Toxic agents like benzene, insecticides
2. Pharmacological agents like antibiotics, anti-inflammatory drugs,
anticonvusltants, antimalarials, oral hypoglycemic agents

Pathophysiology
• In fetus hemapoiesis occurs in liver and spleen and after birth it
continues in bone marrow.
• Within the marrow, the stem cells differentiate into various
types of blood cells.
• Any abnormality of these stem cells leads to ‘pancytopenia’, a
condition in which all three types of blood cells are either
decreased or absent.

Clinical Features
• Increased bruising due to decreased platelet count
• Increased susceptibility to infection due to decreased WBC
count
• Pallor
• Weakness
• Breathing difficulty
• Impaired growth.

• History and physical examination
• Complete blood picture- shows pancytopenia. In severe
Aplastic anemia neutrophil counts are less than 500/ul, platelets
are less than 20,000/ul and reticulocutes are less than 1%
• Bone marrow biopsy: hypocellular marrow.

Management
The goal of management are-
1. to provide symptomatic treatment
2. Restore bone marrow function and
3. Replace pathological bone marrow with normal tissue

i. Symptomatic treatment
• If platelet count is below 10,000 cell/ul, platelet transfusions are
done.
• If anemia is severe, packed cells are given
• If infection occur due to decreased WBC count, antibiotics are
given.

ii. Restoration of bone marrow
function
• Androgenic steroid and corticosteroids are helpful
• Testosterone propionate may be given sublingually or as I/M
injection
• This injection covers fatty hypocellular bone marrow into nearly
normal bone marrow which starts producing cells.
• This therapy should be continued for 2-6months.
• If the disease occurs as a result of autoimmune response, high
doses of Dexamethasone may be used.

iii. Bone marrow transplantation
• For cases not responding to drug therapy, the treatment of
choice is bone marrow transplantation.
• Bone marrow transplantation is done if histocompatible sibling
is available.

NURSING MANAGEMENT
The aim of Nursing intervention is to prevent bleeding and
infection and manage problems related to anemia.
A. Prevention of bleeding:
i. Maintain skin integrity and prevent pressure sores through use of water
mattress or air mattress.
ii. Minimize venipuncture sites by collecting all samples at a time
iii. Avoid intramuscular injection
iv. Check platelet count before any invasive procedure
v. Prevent bleeding from mucus membrane by-
• Keeping mouth clean and free from debris, through use of soft brush or
mouth wash
• Avoid taking rectal temperatures and administration of rectal drugs
• Use of topical thrombin to stop bleeding from lips or nares.

Contd..
B. Prevention of infection
A nurse must take following actions to prevent infection in the
anemic child.
i. Strict hand washing should be practiced by everyone who
comes in contact with the patient
ii. Barrier nursing techniques by everyone who comes in contact
with child
iii. Limit the number of visitors
iv. Isolate the child to prevent contact with other patients having
infection

Contd…
C. Management of problems associated with Anemia:
i. Monitor the child’s hemoglobin level on regular basis
ii. Administer Oxygen if the child is dyspneic
iii. Administer packed cells if prescribed
iv. Monitor growth and development of child
v. Provide adequate rest to child

Introduction
• The term thalassemia is derived from Greek word ‘Thalasa’
meaning ‘sea’.
• It is named so, because the disease had highest incidence
among people living around Mediterranean sea like Italians,
Greeks and Syrians.

Definition
• Thalassemia is a heterogeneous group of inherited chronic
disorders, characterized by absence or decreased synthesis of
one or more globin chain of hemoglobin.
• It is an autosomal recessive disorder.
• The patients show a variable degree of hypochromic anemia,
with evidence of hemolysis and ineffective erythropoiesis.

Diroders of hematologial system

Types
• Hemoglobin is made up of two protein chains- alpha globin and
beta globin.
• Thalassemia occurs when there is defect in the gene that
controls production of these proteins chains.
• Mainly there are two main types-
I. Alpha thalassemia: it occurs when a gene related to alpha
globin protein are missing or changed
II. Beta thalassemia: it occurs when gene defect affects
production of beta globin protein. It is more common than
alpha type.

Depending upon the severity
thalassemia is of two types-
• Thalassemia Minor: it is associated
with decreased beta chain synthesis
and us heterozygous form of disease.
Defective gene is received form one
parent
• Thalassemia Major: it is also known as
‘Cooley’s anemia or Mediterranean
Anemia”. It is homozygous form of
disease. Defective gene is received
form both parents. Persons with
thalassemia major have severe anemia
and all the clinical manifestations of the
disease.

Pathologic defect
• Thalassemia major is associated with little or no capacity to
produce b-chain of hemoglobin.
• When beta chain production is absent or inadequate to meet
the physiological demands of body, marrow expands.
• Large number of RBC’s are produced and will never leave
marrow
• RBC’s that leave marrow will not survive for longer time.
• Marrow expansion continues leading to effective hemoglobin
level, tissue anorexia and repeated infections.
• When Hb level falls below 6gm/dl, cardiac failure occurs.

Clinical features
Diagnosis is usually made in 1st year of life, perhaps as early as 3
months, not more commonly between 10-12 months. clinical
manifestations of thalassemia includes-
• Absent or defective synthesis of hemoglobin
• Inadequate structures RBC’s
• Decreased life span of RBC’s
• Weakness
• Exercise intolerance
• Headache
• Anorexia
• Precordial pain

Repeated hemolysis affects almost all organs of the body in the
following manner-
• Skin: greenish- brown or bronze discoloration of skin.
• Heart: fibrotic changes in myocardium which leads to cardiac
failure
• Spleen: spleenomegaly as a result of rapid destruction of
RBC’s.
• Liver: fibrosis of liver cells which progress to liver fibrosis
• Pancreas: fibrosis of pancreas leading to insulin depended
DM.
• Endocrine system: delayed or absent sexual maturation
• Skeletal system: thinning of cortex, widening of medullary
spaces, osteoporosis, pathological fractures leading to skeletal
deformity, enlarged skull, enlarged face, depressed nasal
bridge, parietal bossing

I. Hemoglobin estimation
II. CBP
III. Peripheral Blood Smear
IV. Bone Marrow Examination
V. Osmotic Fragility
VI. Serum Bilirubin
VII. Serum Iron
VIII.Radiological Studies

Management
Thalassemia can’t be cured but supportive therapy is of value in
maintaining sufficient hemoglobin levels to prevent tissue
hypoxia. Major therapeutic modalities include-
A. Transfusion therapy
B. Chelation therapy
C. spleenectomy

A. BLOOD TRANSFUSION
• Children who cannot maintain hemoglobin level of 7g/dl, should
receive regular transfusions to maintain Hb levels between 10-
12g/dl.
• It is necessary to prevent chronic hypoxemia and to suppress
ineffective erythropoiesis.
• Group and type specific, saline washed, packed red cell
transfusions should be given to the patients at the rate of 10-
15ml/kg every 2-3 weeks.

B. CHELATION THERAPY
• Rapid hemolysis and repeated transfusions results in iron overload which
causes hemochromatosis and hemosiderosis.
• Each unit of transfused blood provides 200mg of elemental iron.
• This excess iron deposits in various organs leading to multiple organ failure
• Chelating therapy is used to excrete excessive iron in urine and creates a
negative iron balance in body.
• The iron status of child should be monitored by repeated serum ferretin
measurements.
• DESFERRIOXAMINE is available as iron chelating agent that may be given
Parenteral route
• It should be given subcutaneously in dose of 40-60mg/kg/ day over a period
of 8-12hrs.
• This therapy should be started from 10th to 15th transfusions.

C. SPLEENECTOMY
• The spleen acts as a store for non-toxic iron, protecting the
body form extra iron, thus early removal of spleen may be
harmful.
• Spleenectomy is justified only in patients with hyperspleenism,
leading to excessive destruction of erythrocytes and increased
need for frequent blood transfusion which results in further iron
accumulation
• This procedure should be considered for the patients who
require more than 200-250ml/kg of packed cells annually.

NURSING MANAGEMENT
I. Early assessment
II. Preparation of child for diagnostic procedures
III. Care during blood transfusion
IV. Administration of chelation therapy
V. Prevention of infection
VI. Education and support of parents and child

Care during blood transfusion
• Blood grouping and cross matching should be ensured before
transfusing the blood
• Blood should be screened for blood borne disorders
• Observe the patient for transfusion reaction causing child,
itching, rash, headache and back pain.
• Always pre-warm the blood before administration
• Monitor vital signs during blood transfusion
• Infuse blood slowly to prevent blood overload
• Follow strict aseptic principles.

Prognosis
• Outcome depends on the severity of disease.
• If the child has thalassemia major, he requires frequent blood
transfusion and chelation therapy
• With chelation therapy child can survive up to 30 years
• Death usually occurs due to cardiac complications during 2nd
decade of life.

Prevention
• Thalassemia screening should be done to find out carrier status
of individuals who are planning to get married as they may give
birth to the same children.
• It can be prevented by marital counseling, prenatal diagnostics
and abortion of affected fetus among thalassemia parents.

DEFINITION
• Hemophilia is genetically transmitted blood clotting disorder,
caused by deficiency of coagulation factor VIII (antihemophilic
factor) or factor IX (christmas factor).

Types
• Depending on the clotting factor involved, hemophilia is of following
types:
i. Hemophilia A or Classic hemophilia: it occurs due to deficiency
of clotting factor VIII. It is most common form of disorder present in
about 1 in 5000-10000 male births
ii. Hemophilia B or christmas disease: it occurs due to deficiency of
clotting factor IX. It occurs in around 1 in 20000-34000 male births
Other less common types are-
iii. Hemophilia C: it occurs due to lack of factor XI
IV. Hageman’s disease: it occurs due to deficiency of factor XII
V. Von Willebrand’s Disease: it is caused by reduced level of von
willebrands factor, a multi proterin thet binds with factor VIII nad
protects it form rapid breakdown within blood

Transmission of disease
• Hemophilia A, B are inherited as sex linked (x) linked recessive
disorder.
• Hemophilia is more likely to occur in males than females
• This is because females have 2 x chromosomes while males have
only one x chromosome, so defective gene is guaranteed to manifest
in any male who carries it.
• Females are always asymptomatic carries f this disorder
• Hemophilia C is an autosomal genetic disorder which can affect both
sexes.

Pathophysiology
• An inherited deficiency of factor VIII or IX alters the activation of intrinsic
clotting pathway.
• As a result children with hemophilia A or B shows manifestations of
prolonged bleeding or delayed clotting.
• The severity of manifestations is proportionate to the severity of factor
deficiency.
• The normal level of factor VIII is 60-140% and of factor IX is 60- 145%.
• A factor level of 30% or less is diagnostic of hemophilia.
• Depending on level of factor deficiency, hemophilia is classified may be
classified as-
1. Mild hemophilia: the level of factor VIII or OIX is 6- 30%
2. Moderate hemophilia: the level of factor VIII or IX is 2-5 %
3. Severe hemophilia: the level of factor VIII or IX is 1% or less.

Clinical features
• Mild hemophilia:
a. Frequent bruises
b. Nose bleeds
c. Bleeding gums
d. Prolonged bleeding after minor surgeries
• Moderate hemophilia:
a. Frequent bleeding episodes
b. Joint bleeding
• severe hemophilia:
a. Bleeding as early as on 1st day of life
b. Excess bleeding after injections
c. Spointaneous bleeding into joint cavities
d. Subcutaneous and intramuscular hemorrhages
e. Intracranial hemorrhages.

• History and clinical features
• Genetic history
• Labaratory tests

Management
• Replacements preparations of factor VIII and IX that are available as
cryoprecipitate made from fresh plasma. One unit of plasma provides 75-125
units of coagulation factors.
• Fresh frozen plasma can also be used to correct. Each ml of plasma
contains 1 unit of factor VIII and slightly less than 1 unit f factor IX.
• Desmopressin: this drug may be used to treat hemophilia. It works by
increasing the amount of clotting factor VIII in child’s blood.

Nursing management
A. Prevention of bleeding
B. Control bleeding
C. Administration of replacement of therapy
D. Education and support to patient and family.

Prevention of bleeding
• Shorten child’s finger nails to aviod scratching
• Avoid using diaper pins in infants
• Provide soft toys whithout sharp corners
• Child should be given soft tooth brush

Control of bleeding
• First aid measures such as-
R- rest: make the child to lie down quietly until bleeding stops
I- ice: apply an ice pack on the bleeding area.
C- compress: apply pressure over the bleeding site. A bleeding joint may be
wrapped with an elastic compression bandage.
E-elevate: position the child so that the bleeding area is raised. Raise the area
above the level of heart if possibel.

Prognosis
• With the advent of factor replacement therapy and with proper care and
support, hemophilics can lead a long and productive life.
• Hemophilics now a days were reaching adulthood with minimal side effects.

DEFINITION
• Purpura is a bleeding disorder characterized by petechiae and
ecchymosis which may be due to either deficiency in number or
quality of platelets or defect in vascular endothelium. Purpura occurs
due to bleeding from capillaries, under the skin.

Types
• Purpura is of 2 types-
1. IDIOPATHIC THROMBOCYTIC PURPURA
2. ANAPHYLACTOID PURPUS

IDIOPATHIC
THROMBOCYTOPENIC
PURPURA

Defintion
• Idiopathic thrombocytopenic purpura, the most
common acquired disorder in children which is
hemorrhagic involving skin, mucus membrane
and internal organs.
• It is most likely an autoimmune response of the
body.
• Its greatest incidence is seen between 3-7
years of age.

Pathophysiology
• Normally the platelets are formed from Megakaryocytes in bone marrow.
• In 60 % cases of Purpura, the cause is autoimmune, with antibodies against
platelets.
• Most often these antibodies are against platelet membrane glycoprotein and
are immunoglobulin type.
• These antibodies destroy platelets leading to platelet deficiency.

Clinicial feature
The child with Purpura may present with-
• Petechiae
• Ecchymosis
• Bleeding form gums
• Epistaxis
• Anemia
• Internal hemorrhage
• Hemarthrosis
• Low grade fever
• Intracranial or intracerebral hemorrhage

• Platelet count: it will be below 20,000/mmcube (1.5-4.5 lakhs/mmcube is
normal)
• Bleeding time and clotting time increased
• Peripheral blood smear- giant forms and stained platelets are seen
• Bone marrow examination: abundant platelet precursors are found.

Management
• Steroids
• Anti D antibodies
• Steroid sparing agents
• Immunoglobulin's
• Platelet infusion

DEFINTION
• Anaphylactoid pupura is a systemic inflammatory disorder, seen
primarily in children between 2-8 years of age. Boys are affected
twice as frequently as girls.

Etiology
• Recent upper respiratory infection history
• Exact etiology is unknown
• Possible precipitating factors include-
1. Drugs like penicillin, aspirin
2. Foods
3. Insect bites
4. Bacterial or viral infections.

Clinical features
• Erythematous petechiae rashes appearing symmetrically over
buttocks and extremities
• GI symptoms include- colicky abdominal pain, vomiting.
Intussusceptions
• Synovial membrane vasculitis
• Renal manifestations like- hematuria, proteinurina, hypertension

• Diagnosed based on typical skin rash
• Joint and renal findings also helps to diagnosis

Management
• Antibiotics are given for specific bacterial infections
• Short term steroid therapy
• Analgesics like acetaminophen to relieve pain.

Nursing management
• Minimize chances of trauma to the child and provide safe environment
• Control bleeding
• Administer prescribed drugs

Prognosis
• The prognosis of purpura is good if adequate supportive care is
given and complications are prevented.
• Most of the patients recover within 2-3 months of onset.

Diroders of hematologial system

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