HEMATOLOGIC DISORDERS

Nursing Care of the Client with Hematologic Disorders

The hematologic system, comprising blood and blood-forming tissues, is vital for body
metabolism. Functions include:

- Transporting oxygen and nutrients to body cells.

- Removing carbon dioxide from cells.

- Initiating blood coagulation.

Hematologic disorders, often termed Blood Dyscrasias, primarily originate in the bone marrow.

Common Disorder in Children:

- Iron Deficiency Anemia is the most prevalent hematologic disorder among children.

ASSESSMENT:

• Nutrition: Note any signs of being a "picky eater," presence of pica, or increased milk intake.

• Past Illnesses: Review past medical history for any relevant illnesses.

• Family History: Assess family history for any hematologic disorders or related conditions.

• Chief Concern: Identify primary complaints such as fatigue, easy bruising, or epistaxis
(nosebleeds).

• Past Medical History: Consider factors like low birth weight or lack of vitamin K administration at
birth.

NURSING DIAGNOSIS:

1. Deficient Knowledge related to the cause of the child’s illness.

2. Imbalanced Nutrition, less than body requirements, related to parental lack of knowledge of the
need for iron-rich foods.

3. Anxiety related to frequent blood-sampling procedures.

4. Pain related to tissue ischemia.

5. Compromised Family Coping related to long-term care needs of a child with a chronic
hematologic disorder.

RED BLOOD CELLS:

• Mature RBCs lack a nucleus, allowing for increased space for oxygen transport but limiting their
lifespan to 120 days.

• RBCs are formed under the stimulation of erythropoietin.

 • RBC’s form first as erythroblast then mature through normoblast and reticulocyte stages to
mature, non-nucleated erythrocytes.

RED BLOOD CELLS

• Production in Infants:

• RBC production occurs primarily in the long bones of the body, which are filled with yellow
marrow.

• Destruction:

• RBCs are destroyed through phagocytosis by reticuloendothelial cells, predominantly located in

the spleen.

• Transition in Childhood:

• In childhood, yellow marrow starts to replace the red marrow in long bones.

• Consequently, blood element production shifts mainly to the flat bones of the body.

• At birth, infants typically have approximately 5 million RBCs per cubic millimeter of blood.

• This count diminishes rapidly in the first few months, reaching a low around 4-5 months of age.

• Subsequently, the RBC count slowly increases until adolescence.

 Hemoglobin Composition:

• Hemoglobin is composed of globin, a protein dependent on nitrogen metabolism, and heme, an

iron-containing pigment facilitating oxygen and carbon dioxide transport.

• Deficiencies in iron stores or nitrogen can disrupt hemoglobin synthesis.

• Hemoglobin levels are highest at birth, decrease around 3 months, then gradually rise until adult
values are reached at puberty.

Special Hemoglobin Types:

• Hemoglobin F (Fetal Hemoglobin):

• Predominant at birth, with a special affinity for oxygen in utero.

• Composed of two alpha and two gamma polypeptide chains.

• Gradually replaced by adult hemoglobin (hemoglobin A) within the first 6 months of life.

• Hemoglobin A:

• Composed of two alpha and two beta chains.

• Diseases affecting beta chains, like sickle cell anemia and Thalassemias, become clinically
apparent after the transition to hemoglobin A.

• Present even in early intrauterine life and can be diagnosed prenatally.

Bilirubin Metabolism:

• Bilirubin is formed from hemoglobin during normal and abnormal erythrocyte destruction by
the reticuloendothelial system. As the portion of heme degraded, it is converted into
protoporphyrin. Protoporphyrin is then further broken down into indirect bilirubin.

• Indirect bilirubin, initially formed, is fat-soluble and converted by the liver into water-soluble
direct bilirubin, which is water soluble and is excreted in bile.

• In newborns, immature liver function may lead to elevated indirect bilirubin levels, manifesting
as jaundice.
Normal Complete Blood Counts:
• RBC:
• Male: 4.7-6.1 million/cm³
• Female: 4.2-5.4 million/cm³
• Hemoglobin:
• Male: 14-18 mg/dL
• Female: 12-16 mg/dL
• Hematocrit:
• Male: 42-52%
• Female: 33-47%
• WBC: 5,000-10,000 cells/cm³
Differential Count:
• Neutrophils: 55-70%
• Lymphocytes: 20-40%
• Monocytes: 2-5%

• Eosinophils: 1-4%

Platelets: 150,000-400,000

These parameters are essential for assessing and monitoring hematologic health and diagnosing any
potential disorders or abnormalities.
ASSESSMENT OF AND THERAPEUTIC TECHNIQUES FOR
HEMATOLOGIC DISORDERS

Bone Marrow Aspiration and Biopsy:

• Provides samples of bone marrow to determine cell type

and quantity.

• Common sites for aspiration: Iliac crests or spines in

children, anterior tibia in neonates.

• Procedure:

• Child positioned prone on a hard treatment table.

• Conscious sedation administered to reduce fear.

• Topical anesthesia applied to reduce pain.

• Site cleaned with antiseptic solution and draped.

• Large-bore needle and stylus inserted into the bone; marrow aspirated.

• Pressure dressing applied to the site.

• Post-procedure care:

• Monitor vital signs until the child is fully awake.

• Check the dressing every 15 minutes.

• Monitor temperature at 12 and 24 hours post-procedure to detect infection.

Blood Transfusion:

• Large blood losses have serious consequences.

• Loss of 15 to 30 percent causes weakness.

• Loss of over 30 percent causes shock, which can be fatal.

• Transfusions are the only way to replace blood quickly, and transfused blood must be of the
same blood group.

Blood Transfusion Reactions:

1. Allergy Reaction: Allergy to protein components of transfusion

• Symptoms: Pruritus, urticaria, wheezing.

• Management:
 • Temporarily discontinue transfusion.

• Administer oxygen as needed.

• Administer antihistamines.

2. Anaphylactic Reaction:

• Symptoms: Headache, chills, back pain, dyspnea, hypotension, hemoglobinuria.

• Management:

• Discontinue transfusion.

• Maintain normal NSS infusion for accessible IV line.

• Provide oxygen.

• Administer diuretics.

3. Circulatory Overload:

• Symptoms: Increased pulse, dyspnea.

• Management:

• Discontinue transfusion.

• Administer oxygen.

• Provide supportive care for pulmonary edema and CHF.

• Administer diuretics.

4. Contaminant Reaction:

• Symptoms: Increased temperature.

• Management:

• Discontinue transfusion.

• Obtain blood culture to rule out bacterial invasion.

5. Hepatitis Reaction: Hepatitis from contaminated transfusion • Symptoms: Fever, jaundice,

lethargy, tenderness over liver.

• Management:

• Investigate transfusion history.

• Refer to Hepatitis care.

6. Hypocalcemia Reaction: Acid-citrate-dextrose anticoagulant in transfusion is combining with

serum calcium and causing hypocalcemia
 • Symptoms: Muscle cramping, twitching of extremities, convulsions.

• Management:

• Discontinue transfusion.

• Administer calcium gluconate IV.

7. Hemosiderosis Reaction: deposition of iron from transfusion in skin Support self-esteem with
altered body imagine

• Symptoms: Bronze-colored skin.

• Management:

• Support self-esteem.

• Administer iron-chelating agent (deferoxamine).

Stem Cell Transplantation:

• Purpose: IV infusion of hematopoietic stem cells to reestablish marrow function in a

child with defective or nonfunctioning bone marrow.

• Source of Stem Cells:

• Obtained from bone marrow via marrow aspiration, or from peripheral or umbilical cord
blood.

• Success Factors:

• Dependent on HLA compatibility of donated donor.

• Types of Transplantation:

• Allogeneic Transplantation: Immune-compatible donor.

• Syngeneic Transplantation: Identical twins.

• Autologous Transplantation: Blood infused after treatment.

• Prevention of Rejection:

• Administer drugs like cylophosphamide (Cytoxan) to suppress marrow and T

lymphocytes production.

Post-Transplantation Care:

• Common Reactions:
 • Fever and chills are common reactions to stem cell transplant infusion.

• Management: Acetaminophen, diazepam, diphenhydramine hydrochloride.

• Monitoring:

• Monitor white blood cell count (WBC).

• Nursing Diagnosis:

• Anxiety related to lack of knowledge about the procedure and expected outcome of
stem cell transplant.

• Outcome Evaluation:

• Ensure parents and child understand they are not responsible for the transplant
outcome.

• Emphasize success factors beyond their control.

• Education of donors and recipients.

Risk Management and Health Promotion:

• Genetic Counseling:

• Ensure families have access to genetic counseling.

• Helps families understand the incidence of inherited disorders and potential risks for
their child.

• Nursing diagnosis

• Risk for delayed growth and development due to extended restrictions and infection
control precautions.

• Outcome evaluation

• Restrict child from interacting with other children to prevent infection.

• Provide sterilized play materials as appropriate.

HEALTH PROMOTION AND RISK MANAGEMENT

• Genetic Counseling:

• Hematologic disorders like sickle cell anemia and hemophilia are often inherited.

• Families should have access to genetic counseling to understand the risk and potential
implications for their child's health.

• Prevention of Iron Deficiency Anemia:

• Iron deficiency anemia, the most common type in children, is preventable.

 • Bottle-fed infants should receive iron-fortified formula for the first year of life.

• Iron-fortified cereals can be introduced to the diet.

• During adolescence, diets low in iron-rich foods may lead to a recurrence of anemia.

• Aplastic Anemia Risk:

• Aplastic anemia, characterized by an inability to produce blood cells, can be acquired

due to exposure to toxic drugs or chemicals.

• Diagnostic Testing:

• All hematologic disorders require blood specimens for diagnosis and ongoing testing for
monitoring and follow-up.

DISORDERS OF RED BLOOD CELLS : Most RBC disorder fall into the category of the anemias, or a
reduction in the number or function of erythrocytes

• Polycythemia

• Polycythemia, characterized by an increase in RBCs, can be as dangerous as a reduction

in RBC production.

• Normochromic, Normocytic Anemia:

• Result of impaired production or loss of erythrocytes without changes in size or color.

• Marked by impaired production of erythrocytes by the bone marrow, or by abnormal or

uncompensated loss of circulating RBCs, as with acute hemorrhage

• Acute Blood-Loss Anemia:

• Causes include trauma, intestinal parasite, acute nephritis, and issues like placenta
previa or premature placental separation in newborns.

• Children are in shock from acute blood loss and appear pale (Pallor). Heart attempts to
compensate by increasing the rate to supplement the needs of the body (Tachycardia).
Decreased oxygen transport causes body cells to register an oxygen deficit (Tachypnea).
Cyanosis due to decreased oxygen transport.

• Oxygen therapy is ineffective due to insufficient RBCs for oxygen transport.

• Usually transient as reduced oxygen levels stimulate erythropoietin release, prompting

bone marrow to increase RBC production.

• Increased reticulocyte count indicates bone marrow response to RBC shortage.

• Anemia of Acute Infection:

• Acute infection or inflammation can increase erythrocyte destruction, leading to

decreased RBC levels.
 • Conditions like osteomyelitis, ulcerative colitis, and advanced renal disease are common.

• Anemia of Renal Disease:

• Reduced erythropoietin production due to kidney dysfunction results in decreased RBC

production.

• Recombinant human erythropoietin administration can correct the anemia but not the
renal disease.

• Anemia of Neoplastic Disease:

• Malignant growths like leukemia impair RBC production by invading bone marrow with
neoplastic cells.

• Aplastic Anemias:
Stem from suppressed hematopoietic activity in the bone marrow affecting WBCs,
platelets, and RBCs.

• Types:

Congenital Aplastic Anemia

Fanconi’s Syndrome

Autosomal recessive trait

Skeletal and Renal Abnormalities

Hypogenitalism

Short stature

Pancytopenia – reduction of all blood cell component

Acquired Aplastic Anemia:

• Decreased bone marrow production due to excessive exposure to radiation, drugs (e.g.,
chloramphenicol, sulfonamides), chemicals (e.g., arsenic, benzene), or insecticides.
• Chemotherapeutic drugs and severe infections can also cause bone marrow dysfunction.
• Serious infection (meningococcal pneumonia)
• Exposure to insecticides also may cause severe bone marrow dysfunction

• Aplastic Anemias

Assessment:

• Lower RBC count (Anemia): Symptoms include pallor, fatigue, and anorexia.
 • Decreased Platelets (Thrombocytopenia): Manifests as bruising, petechiae, epistaxis, and
GI bleeding
• Decrease WBCs (Leukopenia): May result in increased susceptibility to infections Cardiac

Decompensation:

• Tachycardia, tachypnea, shortness of breath, cyanosis, and increased infection risk.

Therapeutic Management:

• Optimal therapy involves STEM CELL Transplantation for both congenital and acquired
aplastic anemia.
• Suppression of T lymphocytes dependent autoimmune response using antihymocyte
globulin (ATG) or cyclosporine, or transfusion of new blood elements.
• Blood transfusion with packed RBCs and platelets to maintain adequate blood elements.
• Administration of oral corticosteroids (Prednisone).
• pTestosterone may be administered to stimulate RBC growth.

DISORDER OF THE RED BLOOD CELLS

• Macrocytic (Megaloblastic) Anemias:

• Characterized by abnormally large immature RBCs.
• Caused by nutritional deficiencies.
• Anemia of Folic Acid Deficiency:
• Combined deficiency of folic acid and vitamin C resulting in enlarged erythrocytes.
• Accompanied by neutropenia and thrombocytopenia.
• Pernicious Anemia (Vitamin B12 Deficiency):
• Vitamin B12 necessary for RBC maturation.
• Results from deficiency or inability to utilize vitamin B12.
• Primarily found in animal sources like cow’s milk and breast milk.
• Assessment:
• Pale appearance, anorexia, irritability, chronic diarrhea.
• Smooth, beefy red tongue due to papillary atrophy.
• Neuropathologic findings may include ataxia, hyporeflexia, paresthesia.
• Management:
• Life-long administration of monthly IM injections of intrinsic factor if due to intrinsic
factor deficiency.
• Hemolytic Anemias:
• Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency:
• RBC destruction depends on the drug and extent of exposure.
• Fever and back pain may accompany marked hemolysis.
• Diagnosis via rapid enzyme screening test or electrophoretic analysis of RBCs.

• Sickle-Cell Anemia:
 • Autosomal recessive disorder affecting the beta chain of hemoglobin.
• RBCs assume abnormally elongated shape.
• Manifests symptoms after fetal hemoglobin changes to adult form (~6 months).
• Diagnosed prenatally via chorionic villi sampling or amniocentesis.
• Neonatal screening identifies hemoglobin S (HbSS) in affected individuals.

• Hemolytic Anemias:
• Sickle-Cell Anemia:
• Hemoglobin Variants:
• Hemoglobin A: Normal adult hemoglobin, consisting of Hb A and Hb A2.
• Hemoglobin C: Abnormal hemoglobin causing hemolytic anemia; often
found with sickle cell disease or thalassemia.
• Hemoglobin F: Normal fetal hemoglobin; reduced after infancy except in
certain hematologic disorders.
• Hemoglobin S: Abnormal hemoglobin leading to sickling and hemolysis
at low oxygen tension; predominant in sickle cell anemia.
• Assessment:
• Diagnosis via hemoglobin electrophoresis at birth to identify sickle cell
anemia.
• Symptoms manifest around six months, including fever, anemia, blood
stasis, and infarction in various body parts.
• Swelling in hands and feet due to aseptic infarction.
• Slight build with long limbs in affected children.
• Protruding abdomen due to spleen and liver enlargement.
• In adults, spleen size may reduce due to repeated infarctions and
atrophy.
• Atrophic spleen increases susceptibility to infections as it loses filtering
function.
• Increased risk of pneumococcal meningitis, salmonella-induced
osteomyelitis, cirrhosis, and reduced kidney function.
• Icteric sclerae due to sickle cell destruction; retinal occlusions may lead
to vision impairment.
• Priapism
•

• Sickle Cell Crisis:

• Sudden, severe onset of sickling.
• Symptoms result from pooling of many new sickled cells in vessels,
causing tissue hypoxia beyond the blockage (vaso-occlusive crisis).
• Triggered by illnesses causing dehydration, respiratory infections, or
strenuous exercise.
• Symptoms are sudden, severe, and painful.
 • Aseptic necrosis of the femur or humerus head may occur with
increased joint pain.

• Laboratory Reports:
• Hemoglobin level below 6 to 8 g/100ml.
• Elevated WBC count (12,000 to 20,000/mm3).
• Increased bilirubin and reticulocyte levels.
• Cerebrovascular accident (CVA) may cause loss of motor
function, coma, seizures, or death.
• Renal involvement such as hematuria or flank pain may occur.
• Acute chest syndrome can lead to death.

• Types of Sickle Cell Crisis:

• Sequestration Crisis:
• Refers to pooling and increased destruction of sickled
cells in the liver and spleen, leading to shock from
hypovolemia.
• Hyperhemolytic Crisis:
• Characterized by increased destruction of RBCs.

• Megaloblastic Crisis:
• Caused by folic acid and vitamin B deficiency.
• Aplastic Crisis:
• Involves a severe sudden decrease in RBC production,
usually from infections.

• •
• Therapeutic Management:
• Three primary needs: Pain relief, adequate hydration, and oxygenation to prevent
further sickling and halt the crisis.
• Hydroxyurea, an antineoplastic agent, has the potential to increase the production of
hemoglobin F. However, it may cause anorexia.
• Nursing Diagnosis:
• Ineffective tissue perfusion related to generalized infarcts due to sickling.
• Outcome Evaluation:
• Normal respiratory rate (RR).
• Arterial blood gas (ABG) at acceptable levels.
• Normal oxygen saturation.
• Urine output greater than 1 mL/kg/h.
• Nursing Management:
• Provide oxygen via nasal cannula and monitor the flow rate carefully.
• Use pulse oximetry to evaluate oxygen saturation levels.
• Encourage bed rest to relieve pain and reduce oxygen expenditure.
• Maintain accurate intake and output monitoring, including urine specific gravity and
hematuria.
• Nursing Diagnosis:
• Ineffective health maintenance related to lack of knowledge regarding long-term needs
of a child with sickle-cell anemia.
• Outcome Evaluation:
• Parent accurately describes the disease process and identifies special precautions
necessary to prevent sickle-cell crisis.
• Management:
• Children who receive blood transfusions should not be given supplementary iron or iron-
fortified formula or vitamins, as they may receive too much iron, leading to
hemochromatosis.
• Regular healthcare visits and childhood immunizations are essential.
• THALASSEMIAS
• Autosomal recessive anemias associated with abnormalities of the beta chain in adult
hemoglobin, most common in the Mediterranean population.
• Thalassemia Minor (Thalassemia Trait):
• Inheritance of recessive genes from only one parent.
• Typically do not display significant symptoms except pallor.
• Thalassemia Major (Homozygous Beta-Thalassemia):
• Also called Cooley’s anemia or Mediterranean anemia.
• Inherit recessive genes from both parents.
• Symptoms do not become apparent until the child’s fetal hemoglobin has largely been
replaced by adult hemoglobin during the second half of the first year of life.

• Pathophysiology:
• Hemoglobin A contains four polypeptide chains: two alpha and two beta, which combine
with four heme complexes to form one Hb molecule.
• In beta thalassemia, the beta chain is defective, resulting in RBCs with less Hb.
• RBCs contain free alpha chains that are unstable and precipitate, destroying RBCs in the
bone marrow.
• Mature and abnormal RBCs that enter the blood are destroyed prematurely in the
spleen, causing further anemia.
• The severe anemia causes the kidneys to produce erythropoietin, leading to ineffective
hematopoiesis versus the rapid destruction of RBCs by the spleen.
 • As a result, the bone marrow becomes hyperplastic, leading to enlargement of the bone
itself (hyperplasia).
• Assessment:
• Inability to bear weight.
• Characteristic changes in the shape of the skull (parietal and frontal bossing) and
protrusion of the upper teeth, with marked malocclusion.
• Broad and flattened base of the nose, and slanted eyes with an epicanthal fold.
• X-ray of bone shows marked osteoporotic tissue, possibly resulting in fractures.
• Hepatosplenomegaly due to excessive iron deposits and fibrotic scarring in the liver, as
well as the spleen's increased attempts to destroy defective RBCs.
• Abdominal pressure from the enlarged spleen may cause anorexia and vomiting.
• Epistaxis is common, as is diabetes mellitus due to pancreatic hemosiderosis, and
cardiac dilatation with an accompanying murmur.
• Arrhythmias and heart failure are frequent causes of death.
• Therapeutic Management:
• Digitalis, diuretics, and a low-sodium diet may be prescribed to prevent heart failure and
myocardial fibrosis.
• Transfusion of packed red blood cells (PRBCs) every 2-4 weeks to maintain hemoglobin
levels.
• Splenectomy may become necessary to reduce the rate of RBC hemolysis and the
number of transfusions needed.
• Bone marrow transplantation can offer a cure.
• With treatment, the overall prognosis is improving but still grave.
POLYCYTHEMIA
• Polycythemia is characterized by an increase in the number of red blood cells (RBCs).
• The condition results from increased erythropoiesis, which occurs as a compensatory
response to insufficient oxygenation of the blood.
• Chronic pulmonary disease and congenital heart disease are major reasons for polycythemia
in children.
• It may also occur due to lower oxygen levels maintained during intrauterine life in newborns
or as a result of transfusion at birth, such as when one twin receives excess blood while the
other twin is anemic.
• Plethora, characterized by a reddened or blackened appearance of the skin, is often observed
due to the excessive amount of blood.
• RBCs are typically macrocytic, and the hemoglobin content is high.
Treatment of Polycythemia involves addressing the underlying cause. Due to the high blood viscosity
resulting from numerous crowded blood cells, there is a risk of cerebrovascular accidents (CVAs) or
emboli. This risk increases particularly if the child becomes dehydrated, such as during fever or
surgery. In severe cases, exchange transfusion may be necessary to reduce the RBC count.

Other Disorders of Red Blood Cells:

• • Neutropenia: Reduced number of white blood cells.
• • Neutrophilia: Increased number of circulating white blood cells, primarily neutrophils.
• Leukemia: Uncontrolled proliferation of white blood cells.
Eosinophilia: Increased number of eosinophils.
 Lymphocytosis: Increased number of lymphocytes.
Platelets:
• Platelets are irregularly shaped disk-like cytoplasmic fragments of a megakaryocyte that are
shed in the marrow sinus and subsequently found in the peripheral blood, where they
function in clotting.
• They contain granules in the central part (granulomere) and clear protoplasm (hyalomere)
peripherally but no definite nucleus, and are about one-third to one-half the size of an
erythrocyte.
PURPURA

Purpura refers to a hemorrhagic rash or small hemorrhages in the superficial layer of the skin. There are
different types, including:
• Idiopathic Thrombocytopenic Purpura: Characterized by a decrease in platelet count due to
immune-mediated destruction of platelets.
• Henoch-Schonlein Syndrome: Also known as IgA vasculitis, it involves inflammation of small
blood vessels and can cause purpura, abdominal pain, arthritis, and kidney involvement.
HEMOPHILIAS

Hemophilias are inherited disorders of blood coagulation.

• HEMOPHILIA A (Factor VIII Deficiency): This is characterized by a deficiency of factor VIII, a
crucial coagulation factor. It is transmitted as a sex-linked recessive trait.
• HEMOPHILIA B (Factor IX Deficiency): Also known as Christmas disease, this form of
hemophilia is less common, affecting only around 15% of people with hemophilia. It involves a
deficiency of factor IX.
• HEMOPHILIA C (Factor XI Deficiency): This form of hemophilia is caused by a deficiency of
factor XI. Symptoms are usually milder compared to hemophilia A and B.
DISSEMINATED INTRAVASCULAR COAGULATION (DIC)
DIC is an acquired disorder of blood clotting that results from excessive trauma or
acute infection. It is
characterized by widespread activation of clotting factors, leading to the formation of small blood clots
throughout the body's blood vessels. This process can consume clotting factors and platelets, resulting
in bleeding tendencies.