Hematology disorder

 Blood formation begins in the 4th week of
gestation.
 During 6months of fetal life liver produce
majority of blood cells and at term bone
marrow carried on this function.

 Blood is composed of RBCs,WBCs,platelets &
plasma.
 Blood is composed of a straw coloured
transparent fluid,plasma, in which different
types of cells are suspended.
 Plasma constitutes about 55% and cells about
45% of blood volume.
 Cellular contents are
erythrocytes,platelets,leukocytes.
 Plasma constitutes of plasma protein,inorganic
minerals,nutrients,wastematerials,hormones,
 gases.

 Albumin-formed in liver,main function to maintain
normal plasma pressure.
 Globulin-formed in liver,remain in lymphoid tissue.
 Clotting factors-essential for coagulation of blood.
 I –fibrinogen
 II-prothrombin
 III-Thromboplastin
 IV-calcium
 V-proaccelerin,Acglobulin
 VII-stable factor,proconvertin
 VIII- antihemophilic factor c

 IX-christmas factor
 X –stuart factor
 XI-Antihemophilic factor c
 XII-Hageman factor
 XIII-fibrin stabilising factor

 INORGANIC SALT:
Involved in muscle contraction,transmission of
nerve impulses,formation of secretion and
maintenance of acid-base balance.
 NUTRIENTS:
To provide energy,heat,minerals to repair and
replacement,and for the synthesis of other blood
components and body secretion.
 WASTE PRODUCTS:
Urea,creatinine and uric acid
formed in liver,conveyed in blood,excreted
through kidney.
 HORMONES:
Influencing cellular activity.

 Blood cell formation is called haemopoiesis and
take place with in red marrow.
Life span in circulation is 120 days.
 Lymphocytes are produced in lymphoid tissue.
Granulocytes
Agranulocytes
 Platelets-non nucleated disc,derived from the
cytoplasm of megakaryocytes.
Life span is between 8 to 11 days.

 Anemia
 Iron deficiency anemia
 sickle cell anemia
 Beta-thalassemia
 Aplastic anemia

 Hemophilia
 Immune thrombocytopenic purpura
 Disseminated intravascular coagulation
 Epistaxis

 Human immunodeficiency virus.
 Severe combined immunodeficiency disease.
 Wiskott-aldrich syndrome.

 Anemia describes a condition in which the number
of red blood cells or the hemoglobin concentration
is reduced below normal values for ages.
RBC-4.5-5.5 million/mm3
HB-11.5-15.5g/dl

 According to National family health survey
Anemia in urban children is 71%,rural is
84%.

 Classification based on Morphology
-microtic
-normocytic
-macrocytic
 Classification based on etiology
-hypochromic
-normochromic
-blood loss, impaired cell production,
increased red cell destruction.

 Decrease in RBCs,Hb or Hct
 Diminished o2 carrying capacity
 Hypoxia
 s/s of anemia

 Dyspnea
 Irritability
 Rapid heart beat
 Easy bruising jaundice
 Pallor of the skin

 History collection
 Physical examination
 CBC
-decreased RBC
-decreased Hg and hematocrit

 Iron medication
 Iron-rich foods to the child’s diet and
restricting milk intake.
 Folic acid and vitamin B12 supplements may
be prescribed.
 Blood transfusions
 Bone marrow transplantation may be
considered in some cases of severe sickle
cell anemia.

 Adequate bed rest
 Prevention of infection

 Monitoring of vitals signs, capillary refill,
color of skin mucous membranes.
 Raising the head position in bed.
 Check and doccument the presence of pain.
 Observation of a delay in verbal
response,confusion or restlessness.
 Maintain the ambient temperature to keep
warm the body needs.

 Provide oxygen as needed.
 Allow the child to eat foods that can be
tolerated, appetite increases.
 Provide food that is accompained by a
nutritional supplement to improve the quality
of nutritional intake.
 Evaluate the child’s weight everyday.

 Iron deficiency anemia is caused by an
inadequate supply iron for normal RBC
formation.This results in smaller RBCs,
depleted RBCs mass,decreased hemoglobin
level, and a decreased oxygen carrying
capacity of the blood.

 It typically occurs between ages 6months
and 3 years.
 Preschool age and adolescence are more
prone to IDA due to discrepency between the
demand and supply of iron for rapid somatic
growth.

 Inadquate dietary iron intake
 Iron malabsorption
 Low iron stores at birth
 Significant blood loss
 Excessive demands for iron required for
growth.

 Iron deficiency anemia occurs in 3 stage
Depletion of hemosiderin,ferritin, and other iron
storage.CBC Counts compounds in the bone
marrow,liver, spleen.
Stage 1

 Lack of transport iron resulting in the
decrease of iron saturation of transfussion.
Stage 2

Marked deficit in transport iron inhibiting the
normal production of hemoglobin.
Stage 3

 The most common clinical features of iron
deficiency anemia is pallor. when
hemoglobin level falls below 5-6gm/dl ,
following features may develop in the child.
• Irritability
• Constipation
• Cardiac enlargement
• Tachycardia

 Weakness
 Dyspnea on exertion
 Reduced alertness
 Poor attention span

 History of the child-dietary history.
 Iron tests will reveal low serum iron capacity,
decreased serum ferritin level and elevated total
iron binding capacity.
 CBC
 Bone marrow study may show absence of
hemosiderin or reduced granules in sideroblasts.

 Improvement of dietary intake,specially iron
and protein containing food should be
emphasized.
 Iron therapy can be administered in oral
route IM,IV route as single total dose
infusion depending upon the child’s
condition.

 Assess for fatigue, activity intolerance, and
other signs of impaired tissue oxygenation.
 Administer prescribed medication or therapy
-oral, parental, transfusion iron.
Iron fortified formula and cereals
-fish, legumes,dired fruits,potatoes,dark green
leafy vegetables,beans,whole grain breads.

 Oral iron supplement-Give the supplements
in two to three divided dose in a small
amount of vit-C containing liquid (orange
juice) between meals to enhance absorption
and minimize adverse effects.
 Administer iron with a dropper to an infant or
through a straw to an older child.

 Explain the potential adverse effect of iron
which include nausea vomiting, diarrhea or
constipation dark green or black stools and
tooth discoloration.

 It is one of a group of disease collectively
termed hemo globinopathies in which normal
hemoglobin(hemoglobin A is partly or
completely replaced by abnormal sickle
hemoglobin Hgbs).
 SCD refers to a group of hereditary disorders
all of which are related to the presence of
Hgbs.

 The disease varies in different geographic
locations.
 Among African-American is about
8%,whereas the frequency of sickle cell trait
is reported to be as high as 40% among
inhabitants of west Africa.

 Autosomal recessive disorder.
 There is a 25% chance f each child having
SCD when both parents carry the trait.

 Abnormal Hbs replaces all or part of normal HbA
 Increased O2 tension and lowered PH
 RBC change from round to sickle or crescent shaped.
o Their angle causes clumping,thrombosis,arterial
obstruction

 Enlarged spleen from congestion with sickle
cell.
 Enlarged and tender liver
 Hematuria
 Inability to concentrate urine
 Enuresis
 Nephrotic syndrome
 Dactylitis

 Chronic villi sampling-reveal SCD prenatally.
 Sickledex screen-will detect the presence of Hbs.
 Hemoglobin electoporesis is needed to distinguish
between the sickle cell trait and the disease.

 Hydration through oral and IV therapy.
 Electrolyte replacement since hypoxia results
metabolic acidosis, which also promotes sickling.
 Analgesics for the pain from vaso occlusion.
 Blood replacement to treat anemia.

 Promote tissue oxygenation.
 Administer appropriate therapeutic
measures.
 Provide oral and Iv hydration fluid to
increase the fluid volume.
 Relieve pain
 Medication include morphine and
hydromorphone
 Position for maximumcomfort.

 Thalassemia is a disease characterized by deficiency
in the rate of production of specific globulin chain in
the hemoglobin.
 The term thalassemia orginated from the Greek word
‘thalassa’ meaning sea.
 It is more common among people living near the
mediteranean.

 In India, it has come with Alexander during
his invasion with Greeks and Egyptians who
settled in India.
 It is more common among north Indians.
 Wide geographical distribution probably as
result of genetic migration through
intermarriage made thalassemia spread to
south India.
 It is classified depending on the HB chain
affected and the amount of globin chain that
is synthesized.

 Normal postnatal hemoglobin(HbA) is composed of
two alpha and two beta chain.
 In beta thalassemia there is partial or complete
deficiency in the synthesis of hemoglobin molecule.

 There is a compensatory increase in the
synthesis alpha chains, and gamma chain
production remain activated in defective
hemoglobin formation.
 This unbalanced polypeptide unit is very
unstable when it disintegrate.

 It dangers the RBCs causing severe anemia.
 For compensation large amount of erythrocytes
is formed unless there is bone marrow
suppression by transfusion therapy.
 Excess iron will be formed by rapid hemolytic
process that will get deposited in various organ
causing hemosiderosis.

 Early signs – unexplained fever
poor feeding
poor weight gain
enlarged spleen
Later signs - chronic hypoxia
damaged to liver, spleen,
heart,pancreas,and lymph glands from
hemochromatosis

• Longer term complications – splenomegaly.
• Skeletal complications –thickened cranial
bones, enlarged head, prominent facial
bones , malocclusions of the teeth.
Cardiac complication – arrhythmias,
pericarditis , heart failure, fibrosis of cardiac
muscle fibers.
Gallbladder disease including gallstones.

 CBC
 HB,HCT
 Reticulocytes count will be decrease
 Fetal blood cells

 Maintain hb level
 Transfusion of blood
 Chelation theraphy- desferal(desferoxamine)
given with vit C
 Children should be protected from
pneumococcal or meningiococcal infection
by immunisation.

 Assess for manifestation and complications.
 Administer blood transfusion –observe for
iron over load and implement chelation
therapy.
 Monitor vital signs.
 Provide information about follow up care

 Aplastic anemia is characterized by
pancytopenia and bone marrow hypoplasia.

 It may be congenital or acquired.
 Primary types include:
fanconi syndrome
black fan-diamond syndrome.
Common causes of acquired aplastic anemia
include:
idiopathic
radiation therapy
drugs

 Lacks of RBCS is characterized by pallor,
lethargy, tachycardia and shortness of breath
on exertion in children, s/s of anemia occur
only when the HB level falls below 5 to 6
g/dl.
 Lack of WBC is characterized by infection.
 Lack of platelets is characterized by
abnormal bleeding,petechiae and bruissing.

 Peripheral blood smear will reveal
pancytopenia.
 Bone marrow aspiration and biopsy are used
to make a definitive diagnosis.

 It is aimed at restoring bone marrow
function.
 Immunosuppressive therapy and
transplantation.

 Prevent infection
 Assess for abnormal bleeding tendency.
 Administer prescribed medication and blood
products.
-antilymphocyte globulin
-antibiotics
monitor for complications of steroid therapy.

IMMUNE THROMBOCYTOPENIC PURPURA:
Immune thrombocytopenic purpura is an
acquired hemorrhagic disorder characterized
by,
 1.Thrombocyopenic ,excessive destruction
of platelets.
 2.Purpura, a discoloration caused by
petechiae beneath the skin.
 3.normal bone marrow with normal or
increased number of immature platelets

(megakaryocytes)and eosinophils.
INCIDENCE:
In children acute or chronic bleeding disorder is
common.
The acute usually occurs between ages 1 to 10
years while chronic form is most common in
children over 9years of age.

1.Upper respiratory tract infections
2.Measles
3.Chicken pox

 Easy bruising and petechiae
 Bleeding from mucous memberane
 Blood in stool or urine
 Hematomas

 CBC-To reveal a platelet count
below20,000/mm3
 Coagulation studies-Tourniquet test,
bleeding time,clotting time.
 Bone marowaspiration

Prevent or minimize bleeding,focus on
safety until the platelet count returns to normal.
 Administer prescribed medications,IV
immunoglobulin(IVG),anti D antibody.
 Monitor child for adverse effects of
steroids,rapid elevation of platelet count.
 Bone marrow aspiration to ruleout leukemia
before administration of prednisone.

 Hemophilia is an inherited bleeding disorder
due to deficiency of plasma coagulation
factors.
 The two most common forms are factor VIII
deficiency(classic hemophilia,hemophilia A)
and factor IX deficiency(christmas
disease,hemophilia B).

 Hereditary hemophilia may account about
80%of cases as sex recessive trait where
abnormal gene carried on the x-
chromosome.
 Affected males may pass the gene to female
offspring making them carrier.
 Female baby may be affected rarely only
when a female carrier bears the child with a
male hemophiliae partner.

 Hemophilia is an x-linked recessive disease
transmitted by females and found
predominantly in males.
 It may also be caused by a gene mutation.

 Hemophilia is classified into three groups;
 HEMOPHILIA A;
It occurs due to deficiency of plasma
factorVIII,anti hemophilic factor(AHF).It
accounts for 80 to 85% of all hemophilias.
 HEMOPHILIA B;[Christmas disease]
It results from deficiency of plasma factor
IX,the plasma thromboplastin
component(PTC).it accounts for about 15 to
20% of cases.

HEMOPHILIA C;
 It results from deficiency of factors XI,plasma
thromboplastin antiplastin anticedent(PTA)it
accounts for few cases only.

Hemophilia is concured with absence or
decrease of deficient function of blood
coagulation factors leading to
excessive,prolonged or delayed bleeding.
The basic deficit is in the intensive phase of the
coagulation process.

The blood clotting factors are essential for the
formation of prothrombin activators which
converts the prothrombin to thrombin.
The rate of prothrombin formation is almost
directly proportional to the amount of prothrombin
activator available.
The rate of clotting factor is proportional to the
amount of formed thrombin.

 Easy bruising
 Prolonged bleeding from wounds
 Spontaneous hematuria
 Epistaxi

 Blood examination shows the following
findings:
1.prolonged clotting time and partial
thromboplastin time.
2.Reduced prothrombin consumption
3.Increased thromboplastin level
4.Bleeding time and prothrombin time are
normal
5.Assay of specific clotting factors
indicates deficient level.x- ray if the affected
joints helps to detect the severity.

 Fresh whole blood transfusion can be given if
the commercially prepared coagulation factors
are not readily available.
 Antifibirinolytics such as aminocaproic acid and
tranexamic acid are given for mucosal bleeding
to prevent clot breakdown by salivary proteins
and to promote hemostatin.
 Synovectomy can be recommended to remove
damage synovium is chronically involved joints.

 ASSESS THE ACUTE OR CHRONIC
BLEEDING:
a.Skin,joints,muscles are assessment priorities.
b.Check vision,hearing and neurologic
development.
c.Check for hematuria and bleeding from the
mouth,lips,gums and rectum.
Administering missing clotting
factor(factorVIII,factorIX).
PREVENT OR MINIMIZE BLEEDING:
Recommand using soft tooth brush,observe the

 Disseminated intravascular coagulation also
known as consumption coagulopathy,is not a
primary disease of coagulation that
complicates a number ofpathologic proess.

 SEVERAL PATHOLOGICAL CONDITIONS:
 Hypoxia
 Acidosis
 Tissue necrosis
 Endothelial damage
 Shock
 Crush injury
 Head injury
 Multiple fracture
 Snake bite
 Burns

 Petechiae
 Purpura
 Bleeding from openings
 Hypotension
 Dysfunction oforgans from infarction and
ischemia.

 History of illness
 Low platelet count
 Diminished fibrinogen level
 Low levelof antithrombin III
 Heparin cofactor II
 Increased level of fibrin split products
 Presence of RBC fragmentation with
schizocytes and reduced PT&PTT.

 Treatment is diverted toward control of the
underlying or initiating cause.
 Administration of platelets and fresh frozen
plasma to replace lost of plasma
components.
 The extremely ill newborn may require
exchange transfusion with fresh blood.
 The administration of IV heparin to inhibit
thrombin formation.

 The goal of nursing care are to be aware of
the possibility of DIC in the severely ill child
and to recognize signs that might indicate its
presence.
 The skills needed to monitor IV infusions and
blood transfusion and administer heparin are
the same as for any child receiving these
therapies.

 Epistaxis also called acute hemorrhage or
nose bleed is a medical condition in which
bleeding occurs from the nasal cavity of the
nostril. Nose bleed can occur due to a
trauma to the nose, due to accident, or injury
to the interior of the nose from pricking.

 In the event of a nosebleed,an essential
intervention is to remain calm.
 To control the bleeding the chid is instructed
to situp and forward to avoid aspiration of
blood.
 Bleeding can be controlled by applying
pressure to the soft lower portion of the nose
with the thumb and forefinger.during this time
child can breaths through the mouth.

 Neoplastic disorders are the leading cause
of death from disease in children past
infancy and almost half of all childhood
cancers invovle the blood forming organs.
Malignant solid tumors of childhood are,
 LEUKEMIAS
 LYMPHOMAS

 Leukemias is a malignant ,increase in the
number of WBC/leukocytes usually all an
immature stage in the bone marrow.most
common in 3 to 5 yearsof age group.
 In the bone marrow,blast cells crowd out
healthy WBC/RBC and platelets leading to
bone marrow defusion.

 Acute myeloid (or myelogenous) leukemia
(AML)
 Chronic myeloid (or myelogenous) leukemia
(CML)
 Acute lymphocytic (or lymphoblastic)
leukemia (ALL)

 Anemia
 Weakness
 Pallor dyspnea
 Bleeding
 Petechiae
 Infection
 Fever
 Malaise
 Enlarged lymphnodes
 Abdominal pain

 Induction(combination of radiation and
intrathecal chemotherapy)
 CNS prophylaxis to prevent CNS disease.
 Maintance chemotherapy for 2-3 years.
 DIAGNOSIS:
 Blood studies
 Bone marrow biopsy.

 Provide care for child receiving
chemotherapy&radiation.
 Provide support for child/family.needs will
change as treatment progress.
 Administer sedation prior to procedure as
ordered.
 Support child during painful procedures.
 Use distention guided imagery.
 Allow child to retain as much control as
possible.

 Lymphoma is cancer that begins in
infection-fighting cells of the immune system,
called lymphocytes. These cells are in the
lymph nodes, spleen, thymus, bone marrow,
and other parts of the body. When you
have lymphoma, lymphocytes change and
grow out of control.
 1.HODGKINS LYMPHOMA
 2.NON HODGKINS LYMPHOMA

 Since the first causes of AIDS were identified
in the early 1980.
 HIV infection has generated intense medical
investigation.
 Research has led to early diagnosis and
improved medical treatments for HIV
infection,changing this disease from a rapidly
fatal one to a chronic but terminal disease of
childhood.

 More than 90% of these children who
received blood products.more than acquired
the disease perinatally from their mothers.
 Smaller number of children were infected
through the transfusion of contaminated
blood or blood products.

 HIV is a retro virus that is transmitted by
lymphocytes and monocytes.
 Horizontal transmission of HIV occurs
through intimate sexual contact or parenteral
exposure to blood or body fluids containing
visible blood.
 Perinatal transmission occurs when a HIV
infected pregnant women passes the
infection to the infant.

HIV virus primarily injects a specific subset of T
lymphocytes,the CD4+ T cells
Virus takes over the machinery of the CD4+
lymphocytes,using it to replicate
itself,rendering the CD4+ cell dysfunctional

Lymphocyte count gradually decreases over
time,leading to progressive immuno deficiency
Count reaches critical level
Risk of opportunistic illness followed by death

HIV INFECTION:
 Lymphadenopathy
 Hepatospleenomegaly
 Oral candidiasis
 Chronic or recurrent diarrhoea
 Failure to thrive
 Parotitis
 Developmental delay

CLINICAL MANIFESTATION OF AIDS IN
CHILDREN:
 Pneumocystis carinii pneumoniae
 Wasting syndrome
 Recurrent bacterial infection
 HIV encephalopathy
 Herpes simplex disease
 cryptosporidiasis

Hematology disorder

More Related Content

What's hot

Similar to Hematology disorder

Recently uploaded

Hematology disorder