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Hematology

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RBC's travel through the body delivering oxygen and removing waste. ... Symptoms: rash, urticaria, respiratory distress, or anaphylaxis. Interventions: ... – PowerPoint PPT presentation

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Title: Hematology


1
Hematology
  • Jan Bazner-Chandler
  • CPNP, CNS, MSN, RN

2
Blood
  • Blood is the fluid of life
  • Blood is composed of
  • Plasma
  • RBC
  • WBC
  • Platelets

3
Plasma
  • Plasma consists of
  • 90 water.
  • 10 solutes albumin, electrolytes and
    proteins.
  • Proteins consist of clotting factors, globulins,
    circulating antibodies and fibrinogen.

4
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.

5
RBCs
  • Average life cycle is 120 days.
  • The bones are continually producing new cells.

6
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.

7
WBCs
  • WBC life span is from a few days to a few weeks.
  • WBCs will increase when fighting infection.

8
Platelets
  • Platelets are irregularly-shaped, colorless
    bodies that are present in blood.
  • Their sticky surface lets them form clots to stop
    bleeding.

9
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

10
Hematocrit
  • Normal hematocrit levels are 35 to 44.
  • Panic levels
  • Hmct less than 15
  • Hmct greater than 60

11
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.

12
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

13
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.

14
Bone Marrow Aspiration
15
Treatment Modalities
  • Transfusion
  • Packed red blood cells anemia
  • Platelets platelet dysfunction
  • Fresh frozen plasma coagulation factors

16
Blood Transfusions
  • 3 types of transfusion reactions
  • Hemolytic
  • Allergic
  • Febrile

17
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.

18
Hemolytic reaction
  • Flank pain
  • Fever
  • Chills
  • Bloody urine
  • Rash
  • Low blood pressure
  • Dizziness / fainting

19
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

20
Febrile Reaction
  • Often occurs after multiple blood transfusions.
  • Symptomsfever, chills, and diaphoresis.
  • Interventions
  • Slow transfusion and administer antipyretic.
  • Administer antipyretic prior to administration.

21
Allergic Reaction
  • Symptoms rash, urticaria, respiratory distress,
    or anaphylaxis.
  • Interventions
  • administer antihistamine before transfusion
  • Physician may order washed rbcs

22
Hematologic Conditions
23
Alteration in Hematologic Status
  • Disorders of hemostasis or clotting factors
  • Structural or quantitative abnormalities in the
    hemoglobin.
  • Anemias
  • Aplastic Anemia

24
Genetic Implications
  • The following have a genetic link implications
    for genetic screening and fetal diagnosis
  • Sickle cell anemia
  • Thalassemia
  • Hemophilia

25
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

26
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

27
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

28
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.

29
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.

30
Symptoms
  • Circumcision may produce prolonged bleeding.
  • As child matures and becomes more active the
    incidence of bleeding due to trauma increases

31
Symptoms
  • May be mild, moderate or severe
  • Bleeding into joint spaces, hemarthrosis
  • Most dangerous bleed would be intracranial.

32
Diagnosis
  • Presenting symptoms
  • Prolonged activated aPTT and decreased levels of
    factor VIII or IX.
  • Genetic testing to identify carriers

33
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.

34
Nursing Diagnoses
  • Risk for injury
  • Pain with bleed especially into a joint
  • Impaired physical mobility
  • Knowledge deficit regarding disease and
    management of disease

35
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.

36
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.

37
Family Education
  • Medic-Alert bracelet
  • Injury prevention appropriate for age
  • Signs and symptoms of internal bleeding or
    hemarthrosis
  • Dental checkups
  • Medication administration

38
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.

39
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.

40
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.

41
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.

42
Nursing Diagnoses
  • Altered tissue perfusion
  • Risk for injury
  • Anxiety

43
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.

44
Prognosis
  • Depends on the underlying disorder and the
    severity of the DIC.

45
ITP
  • Idiopathic thrombocytopenic purpura
  • Idiopathic cause is unknown
  • Thrombocytopenic blood does not have enough
    platelets
  • Purpura excessive bleeding / bruising

46
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

47
Symptoms
  • Random purpura
  • Epistaxis, hematuria, hematemesis, and
    menorrhagia
  • Petechiae and hemorrhagic bullae in mouth

48
Diagnostic Tests
  • Low platelet count
  • Peripheral blood smear
  • Antiplatelet antibodies
  • Normal platelet count 150,000 to 400,000

49
Management
  • IV gamma globulin to block antibody production,
    reduce autoimmune problem
  • Corticosteroids to reduce inflammatory process
  • IV anti-D to stimulate platelet production

50
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

51
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.

52
Whaley Wong Text
53
Crescent Shaped Cells
54
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

55
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

56
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

57
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

58
Nursing Diagnoses
  • Altered tissue perfusion
  • Pain
  • Risk for infection
  • Knowledge deficit regarding disease process

59
Nursing Management - Hospital
  • Increase tissue perfusion
  • Oxygen
  • Blood transfusion if ordered
  • Bed rest
  • Pain management
  • Hydration
  • IV fluids as ordered
  • Oral intake of fluids

60
Nursing Management
  • Adequate nutrition
  • Emotional Support
  • Discharge instructions
  • Information about disease management
  • Daily folic acid
  • Control of triggers
  • Prophylactic antibiotics
  • Immunizations / Pneumococcal

61
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

62
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

63
RBC Characteristics
  • Microcytosis small in size
  • Hypochromia decrease hemoglobin
  • Poikilocytosis abnormal shape

64
Treatment / Prognosis
  • Supportive
  • Blood transfusions as needed
  • Bone marrow transplant

Poor prognosis / death within 1st year due to
septicemia or heart failure.
65
Iron Deficiency Anemia
  • Most common nutritional deficiency
  • Depletion of iron stores

66
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

67
IDA
  • Occurs in children experiencing
  • Rapid physical growth
  • Low iron intake
  • Inadequate iron absorption
  • Loss of blood

68
Symptoms
  • Associated with low oxygenation of tissue
  • Pallor
  • Fatigue
  • Shortness or breath
  • Irritability
  • Intolerance of physical work / exercise

69
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

70
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

71
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.

72
Causes
  • Exposure to drugs
  • Exposure to chemicals
  • Exposure to toxins
  • Infection
  • Idiopathic in nature

73
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.
74
Management
  • Immunosuppressive therapy
  • Antithymocyte globulin
  • Administered IV over 4 days
  • Response seen within 3 months
  • Bone Marrow Transplant

75
Hyper-bilirubinemia
76
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.

77
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

78
Photo Therapy
79
Fiberoptic Blanket
80
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
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