Hematology

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Hematology

• Jan Bazner-Chandler
• CPNP, CNS, MSN, RN

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Blood

• Blood is the fluid of life
• Blood is composed of
• Plasma
• RBC
• WBC
• Platelets

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Plasma

• Plasma consists of
• 90 water.
• 10 solutes albumin, electrolytes and
  proteins.
• Proteins consist of clotting factors, globulins,
  circulating antibodies and fibrinogen.

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Red Blood Cells

• RBCs travel through the body delivering oxygen
  and removing waste.
• RBCs are red because they contain a protein
  chemical called hemoglobin which is bright red in
  color.
• Hemoglobin contains iron, making it an excellent
  vehicle for transporting oxygen and carbon
  dioxide.

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RBCs

• Average life cycle is 120 days.
• The bones are continually producing new cells.

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White Blood Cells

• The battling blood cells.
• The white blood cells are continually on the look
  out for signs of disease.
• When a germ appears the WBC will
• Produce protective antibodies.
• Surround it and devour the bacteria.

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WBCs

• WBC life span is from a few days to a few weeks.
• WBCs will increase when fighting infection.

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Platelets

• Platelets are irregularly-shaped, colorless
  bodies that are present in blood.
• Their sticky surface lets them form clots to stop
  bleeding.

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Blood Values

• CBC with differential and platelet count.
• Hgb
• Normal levels are 11 to 16 g / dl
• Panic levels are
• Less than 5 g / dl
• More than 20 g / dl

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Hematocrit

• Normal hematocrit levels are 35 to 44.
• Panic levels
• Hmct less than 15
• Hmct greater than 60

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Hemoglobin and Hematocrit

• Can be used as a simple blood test to screen for
  anemia.
• The CBC with differential would be used to help
  diagnose a specific disorder.
• A bone marrow aspiration would be the most
  conclusive in determining cause of anemia
  aplastic / leukemia.

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Bone Marrow

• Bone marrow is the spongy substance found in the
  center of the bones.
• It manufactures bone marrow stem cells, which in
  turn produce blood cells.
• Red blood cells carry oxygen to tissue
• Platelets help blood to clot
• White blood cells fight infection

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Bone Marrow Transplant

• Donor is placed under anesthesia.
• Marrow is aspirated out of the iliac crest.
• Marrow is filtered and treated to remove bits of
  bone and other unwanted cells and debris,
  transferred to a blood bag, and is infused into
  the patients blood just like at transfusion.

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Bone Marrow Aspiration
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Treatment Modalities

• Transfusion
• Packed red blood cells anemia
• Platelets platelet dysfunction
• Fresh frozen plasma coagulation factors

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Blood Transfusions

• 3 types of transfusion reactions
• Hemolytic
• Allergic
• Febrile

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Hemolytic Reaction

• Refers to an immune response against transfused
  blood cells.
• Antigens, on the surface of red blood cells, are
  recognized as foreign proteins and can
  stimulate B lymphocytes to produce antibodies to
  the red blood cell antigens.

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Hemolytic reaction

• Flank pain
• Fever
• Chills
• Bloody urine
• Rash
• Low blood pressure
• Dizziness / fainting

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Nursing Management

• Stop the blood transfusion.
• Start normal saline infusion.
• Take vital signs with blood pressure
• Call the MD
• Obtain blood sample and urine specimen.
• Return blood to blood bank.
• Document

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Febrile Reaction

• Often occurs after multiple blood transfusions.
• Symptomsfever, chills, and diaphoresis.
• Interventions
• Slow transfusion and administer antipyretic.
• Administer antipyretic prior to administration.

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Allergic Reaction

• Symptoms rash, urticaria, respiratory distress,
  or anaphylaxis.
• Interventions
• administer antihistamine before transfusion
• Physician may order washed rbcs

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Hematologic Conditions
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Alteration in Hematologic Status

• Disorders of hemostasis or clotting factors
• Structural or quantitative abnormalities in the
  hemoglobin.
• Anemias
• Aplastic Anemia

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Genetic Implications

• The following have a genetic link implications
  for genetic screening and fetal diagnosis
• Sickle cell anemia
• Thalassemia
• Hemophilia

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Bleeding Disorders

• Three types Hemophilia males only
• Type A most common factor VIII deficiency
• Type B - lack of factor IX (Christmas Disease)
• Type C lack of factor XI
• Von Willebrand Disease 1 of population men
  or women prolonged bleeding time

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Hemophilia Type A

• Hemophilia type A is the deficiency of clotting
  factor VIII.
• A serious blood disorder
• Affects 1 in 10,000 males in the US
• Autoimmune disorder with lowered level of
  clotting factor
• All races and socio economic groups affected
  equally

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Hemophilia

• Hemophilia is a sex-linked hereditary bleeding
  disorder
• Transmitted on the X chromosome
• Female is the carrier
• Women do not suffer from the disease itself

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Historical Perspective

• First recorded case in Talmud Jewish text by an
  Arab physician documentation of two brothers
  with bleeding after circumcision.
• Queen Victoria is carrier and spread the disease
  through the male English royalty.

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Goals of Care

• Goals of care
• Provide factor VIII (IX) to aid blood in
  clotting.
• To decrease transmission of infectious agents in
  blood products hepatitis AIDS.
• Future gene therapy to increase production of
  clotting factor.

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Symptoms

• Circumcision may produce prolonged bleeding.
• As child matures and becomes more active the
  incidence of bleeding due to trauma increases

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Symptoms

• May be mild, moderate or severe
• Bleeding into joint spaces, hemarthrosis
• Most dangerous bleed would be intracranial.

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Diagnosis

• Presenting symptoms
• Prolonged activated aPTT and decreased levels of
  factor VIII or IX.
• Genetic testing to identify carriers

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Treatment

• Products used to treat hemophilia are
• Fresh frozen plasma and cryoprecipitate which are
  from single blood donors and require special
  freezing.
• Second generation of factor VIII are made with
  animal or human proteins.

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Nursing Diagnoses

• Risk for injury
• Pain with bleed especially into a joint
• Impaired physical mobility
• Knowledge deficit regarding disease and
  management of disease

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Nursing interventions

• No rectal temps.
• Replace the factor as ordered by physician.
• Manage pain utilizing analgesics as ordered.
• Maintaining joint integrity during acute phase
  immobilization, elevation, ice.
• Physical therapy to prevent flexion contraction
  and to strengthen muscles and joints.
• Provide opportunities for normal growth and
  development.

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Teaching

• Avoid aspirin which prolongs bleeding time in
  people with normal levels of factor VIII.
• A fresh bleeding episode can start if the clot
  becomes dislodged.
• Natural reactions in the body cause the clot that
  is no longer needed to break down. This process
  occurs 5 days after the initial clot is formed.

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Family Education

• Medic-Alert bracelet
• Injury prevention appropriate for age
• Signs and symptoms of internal bleeding or
  hemarthrosis
• Dental checkups
• Medication administration

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Long Term Complications

• 20 develop neutralizing antibodies that make
  replacement products less effective.
• Gene therapy providing continuous production of
  the deficient clotting factor could be the next
  major advance in hemophilia treatment.

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Disseminated Intravascular Coagulation or DIC

• DIC is an acquired coagulopathy that is
  characterized by both thrombosis and hemorrhage.
• DIC is not a primary disorder but occurs as a
  result of a variety of alterations in health.

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Assessment

• The most obvious clinical feature of DIC is
  bleeding.
• Renal involvement hematuria, oliguria, and
  anuria.
• Pulmonary involvement hemoptysis, tachypnea,
  dyspnea and chest pain.
• Cutaneous involvement petechiae, ecchymosis,
  jaundice, acrocyanosis and gangrene.

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Management of DIC

• Treatment of the precipitating disorder.
• Supportive care with administration of platelet
  concentration and fresh frozen plasma and
  coagulation factors.
• Administration of heparin (controversial in
  children).
• Heparin potentates anti-thrombin III which
  inhibits thrombin and further development of
  thrombosis.

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Nursing Diagnoses

• Altered tissue perfusion
• Risk for injury
• Anxiety

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Nursing Interventions

• Rigorous ongoing assessment of all body systems
• Monitor bleeding
• No rectal temps
• Avoid trauma to delicate tissue areas
• All injections sites and IV sites need to be
  treated like an arterial stick.

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Prognosis

• Depends on the underlying disorder and the
  severity of the DIC.

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ITP

• Idiopathic thrombocytopenic purpura
• Idiopathic cause is unknown
• Thrombocytopenic blood does not have enough
  platelets
• Purpura excessive bleeding / bruising

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Immune Thrombocytopenic Purpura

• Antibodies destroy platelets
• Antibodies see platelets as bacteria and work to
  eliminate them
• ITP is preceded by a viral illness
• URI
• Varicella / measles vaccine
• Mononucleosis
• Flu

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Symptoms

• Random purpura
• Epistaxis, hematuria, hematemesis, and
  menorrhagia
• Petechiae and hemorrhagic bullae in mouth

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Diagnostic Tests

• Low platelet count
• Peripheral blood smear
• Antiplatelet antibodies
• Normal platelet count 150,000 to 400,000

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Management

• IV gamma globulin to block antibody production,
  reduce autoimmune problem
• Corticosteroids to reduce inflammatory process
• IV anti-D to stimulate platelet production

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Sickle Cell Anemia

• Autosomal recessive disorder
• Defect in hemoglobin molecule
• Cells become sickle shaped and rigid
• Lose ability to adapt shape to surroundings.
• Sickling may be triggered by fever and emotional
  or physical stress

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Pathophysiology

• When exposed to diminished levels of oxygen, the
  hemoglobin in the RBC develops a sickle or
  crescent shape the cells are rigid and obstruct
  capillary blood flow, leading to congestion and
  tissue hypoxia clinically, this hypoxia causes
  additional sickling and extensive infarctions.

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Whaley Wong Text
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Crescent Shaped Cells
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Body Systems Affected by SS

• Brain CVA paralysis - death
• Eyes retinopathy blindness
• Lungs pneumonia
• Abdomen pain, hepatomegaly, splenomegaly
  (medical emergency due to possible rupture
• Skeletal joint pain, bone pain osteomyelitis
• Skin chronic ulcers poor wound healing

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Vaso-occlusive Crisis

• Stasis of blood with clumping of cell in the
  microcirculation, ischemia, and infarction
• Most common type of crisis painful
• Signs include fever, pain, tissue engorgement

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Splenic Sequestration

• Life-threatening / death within hours
• Pooling of blood in the spleen
• Signs include profound anemia, hypovolemia, and
  shock
• Abdominal distention, pallor, dyspnea,
  tachycardia, and hypotension

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Aplastic Crisis

• Diminished production and increased destruction
  of red blood cells
• Triggered by viral infection or depletion of
  folic acid
• Signs include profound anemia, pallor

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Nursing Diagnoses

• Altered tissue perfusion
• Pain
• Risk for infection
• Knowledge deficit regarding disease process

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Nursing Management - Hospital

• Increase tissue perfusion
• Oxygen
• Blood transfusion if ordered
• Bed rest
• Pain management
• Hydration
• IV fluids as ordered
• Oral intake of fluids

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Nursing Management

• Adequate nutrition
• Emotional Support
• Discharge instructions
• Information about disease management
• Daily folic acid
• Control of triggers
• Prophylactic antibiotics
• Immunizations / Pneumococcal

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Patient Education

• Necessity of following plan of care
• Signs and symptoms of impending crisis.
• Signs and symptoms of infection
• Preventing hypoxia from physical and emotional
  stress
• Proving adequate rest

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Beta-Thalassemia

• Hereditary / autosomal defect
• Genetic defect on chromosome 11
• Mediterranean descent
• Defect in the beta globin gene
• Beta globin chains are required for synthesis of
  hemoglobin A

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RBC Characteristics

• Microcytosis small in size
• Hypochromia decrease hemoglobin
• Poikilocytosis abnormal shape

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Treatment / Prognosis

• Supportive
• Blood transfusions as needed
• Bone marrow transplant

Poor prognosis / death within 1st year due to
septicemia or heart failure.
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Iron Deficiency Anemia

• Most common nutritional deficiency
• Depletion of iron stores

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Abnormal Laboratory Values

• Hemoglobin levels less than 8 g/dL
• Decreased levels of Serum Iron or Total Iron
  Binding or Serum Ferritin
• Microcytic and hypochromic red blood cells

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IDA

• Occurs in children experiencing
• Rapid physical growth
• Low iron intake
• Inadequate iron absorption
• Loss of blood

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Symptoms

• Associated with low oxygenation of tissue
• Pallor
• Fatigue
• Shortness or breath
• Irritability
• Intolerance of physical work / exercise

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Management

• Iron supplementation
• Given in a.m. on an empty stomach
• To avoid staining of teeth, give using a syringe,
  dropper or straw
• Instruct caretaker that child may have
  dark-colored stools

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Management

• Nutritional counseling
• Infants younger than 12 months should be on
  formula until around 12 months of age
• Infants 12 months or older
• Decrease intake of milk
• Introduce solid foods
• Children iron fortified cereals, foods, meat,
  green leafy vegetables
• Teenagers reduce junk food

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Aplastic Anemia

• Acquired or inherited
• Normal production of blood cells in the bone
  marrow is absent or decreased.
• A marked decrease in RBCs, WBCs and platelets.

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Causes

• Exposure to drugs
• Exposure to chemicals
• Exposure to toxins
• Infection
• Idiopathic in nature

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Blood Characteristics

• Neutophil less than 500
• Platelet less than 20,000
• Hemoglobin less than 7
• Reticulocytes 1
• Nursing Diagnosis?

Bone marrow reveals hypo-cellular and fatty
marrow.
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Management

• Immunosuppressive therapy
• Antithymocyte globulin
• Administered IV over 4 days
• Response seen within 3 months
• Bone Marrow Transplant

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Hyper-bilirubinemia
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Hyperbilirubinemia

• Many babies have some jaundice. When they are a
  few days old, their skin slowly begins to turn
  yellow. The yellow color comes from the color of
  bilirubin. When red blood cells die, they break
  down and bilirubin is left. The red blood cells
  break down and make bilirubin. In newborns, the
  liver may not be developed enough to get rid of
  so much bilirubin at once. So, if too many red
  blood cells die at the same time, the baby can
  become very yellow or may even look orange. The
  yellow color does not hurt the baby's skin, but
  the bilirubin goes to the brain as well as to the
  skin. That can lead to brain damage.

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Signs and symptoms

• Very yellow or orange skin tones (beginning at
  the head and spreading to the toes)
• Increased sleepiness, so much that it is hard to
  wake the baby
• High-pitched cry 
• Poor sucking or nursing 
• Weakness, limpness, or floppiness

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Photo Therapy
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Fiberoptic Blanket
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Nursing Interventions

• Monitor bilirubin levels
• Assess activity level muscle tone infant
  reflexes
• Encourage po intake May need to supplement with
  formula if inadequate breastfeeding
• Weight daily to assess hydration status
• Monitor stools amount and number
• Cover eyes while under bili-lights
• Facilitate parent - infant bonding
• Loss of moro or startle reflex can indicate
  possible brain damage due to Kernicterus