1. Hematologic Disorders
Chapter 32
Hematologic Disorders 1

2. Learning Objectives
List the components of the hematologic system and describe
their role in oxygenation and hemostasis.
Identify data to be collected when assessing a patient
with a disorder of the hematologic system.
Describe tests and procedures used to diagnose disorders
of the hematologic system and nursing considerations
for each.
Describe nursing care for patients undergoing common
therapeutic measures for disorders of the hematologic
system.
Describe the pathophysiology, signs and symptoms,
medical diagnosis, and medical treatment for selected
disorders of the hematologic system.
Assist in planning nursing care for a patient with a disorder
of the hematologic system.

3. Anatomy and Physiology of the Hematologic System3

4. Structures and Components of the Hematologic System
Bone marrow
Liver
Spleen
Blood
Red blood cells (erythrocytes)
Platelets (thrombocytes)
Clotting factors
Plasma
Where are the majority of red blood cells and platelets produced?
The bone marrow is the spongy center of the bones where the red blood cells and platelets are made.
The liver manufactures clotting factors.
The spleen removes old red blood cells from circulation.
Blood transports oxygen from the lungs to tissues and maintains hemostasis.
Hemoglobin in the red blood cells makes the transport of oxygen and carbon dioxide possible.
Platelets activate the blood clotting system by going to a break in a blood vessel and forming a platelet plug.
The clotting factors form a stable fibrin matrix over the wounded area, protecting the injured site while the healing process is completed.
Plasma carries the red blood cells, platelets, and clotting factors through the circulatory system. 4

5. Figure 32-1 5

6. Physiologic Functions of the Hematologic System
Oxygenation
Red blood cells transport oxygen from the lungs to the tissues and carbon dioxide from the tissues back to the lungs for excretion
Hemoglobin in red blood cells combines with oxygen and carbon dioxide to accomplish oxygenation
What is the coagulation cascade? 6

7. Physiologic Functions of the Hematologic System
Hemostasis
Control of bleeding
Blood vessel constricts; reduces bleeding
Platelets adhere to the injured blood vessel, forming an unstable platelet plug
Coagulation initiated, forming a stable fibrin matrix, (scab) 7

8. Figure 32-2 8

9. Age-Related Changes Bone marrow becomes less productive
Hematologic function not affected unless a person is unusually stressed with trauma, a chronic illness, or treatment for cancer
In conditions necessitating a higher production of blood cells, bone marrow usually responds to the increased demand, given time 9

10. Nursing Assessment of the Hematologic System10

11. Chief Complaint and History of Present Illness
Inquire about bruising easily, bleeding for an unusually long time, or chronic fatigue
May be the symptoms of an underlying hematologic disorder 11

12. Past Medical History
Cancer or prior cancer treatment, human immunodeficiency virus (HIV) infection, liver disease, kidney disease, malabsorption disease, prolonged bleeding or delayed healing with surgery or dental extractions, a history of blood transfusion, prosthetic heart valves, or indwelling venous access device, indicating that the patient needed long-term venous access
Note any history of blood transfusions
Medications used or recent change in medication
Why is it important to find out what over-the-counter medications the patient is taking? 12

13. Family History Sickle cell disease or hemophilia
Death of a family member at a young age for reasons other than trauma may indicate a genetic hematologic disorder 13

14. Review of Systems
Changes in skin color, skin dryness, pruritus (itching), and brittle fingernails or toenails
Dizziness, vertigo, confusion, and pain
Headaches
Changes in vision
Epistaxis, hemoptysis, dyspnea, heart palpitations, or chest pain
Changes in eating habits, including appetite or episodes of nausea or vomiting
What characteristics of a patient's headache should be assessed? 14

15. Review of Systems Bleeding or pain in the mouth, gums, or tongue
Normal bowel function and recent changes
Blood in the urine
Unusually heavy menses
Joint pain
Fatigue or cold intolerance 15

16. Functional Assessment
Occupation and hobbies
Self-concept
Activity and exercise
Sleep and rest
Interpersonal relationships
Coping and stress
Perception of health
How can a patient's occupation or hobbies affect the hematologic system? 16

17. Physical Examination Measure vital signs, height, and weight
General survey
Patient’s responsiveness, mood, expression, and posture
Any reddened, swollen, or painful areas
Skin
Note general color
Describe bruising
Head and neck
Signs of bleeding in eyes, ears, nose, mouth, throat
Cracking at the corners of the mouth
What variations in the vital signs may indicate a hematologic disorder?
A patient with a low red blood cell count may appear pale. 17

18. Physical Examination Thorax Lungs Heart and vascular system Abdomen
Assess respiratory rate and effort
Auscultate breath sounds
Heart and vascular system
Assess heart rate, resting blood pressure, and adaptation of blood pressure to position changes
Abdomen
Inspect/palpate the abdomen for distention and tenderness
If stool specimen available, guaiac test for microscopic blood
If patient can provide urine sample, it can be tested for blood
Patients with low red blood cell counts often are dyspneic, or short of breath, because they do not have enough red blood cells to carry oxygen to all their tissues.
What causes the heart rate to increase in a patient with a low red blood cell count?
The liver and spleen can become enlarged with blood cell disorders, causing abdominal fullness and tenderness. 18

19. Diagnostic Tests and Procedures
Blood tests
Red blood cell count
Hemoglobin (Hb or Hgb)
Hematocrit (Hct)
Platelet counts
Bone marrow biopsy
The red blood cell count is the total number of red blood cells found in a cubic millimeter (mm3) of blood.
The hemoglobin indicates the oxygen-carrying capacity in the blood.
The hematocrit is the percentage of red blood cells in whole blood.
Normal platelet counts range from 140,000 to 440,000 platelets/mm3 of blood. 19

20. Common Therapeutic Measures20

21. Therapeutic Action
Nursing actions for the patient at Risk for Injury from low red blood cell counts
See Box 32-3, p. 575 21

22. Therapeutic Action
Nursing actions for the patient at Risk for Injury from bleeding
See Box 32-4, p. 579 22

23. Blood Product Transfusion
Typing for transfusions
Sample sent for typing and crossmatching
Type A, B, AB, or O; either Rh positive or Rh negative
O negative: universal donors because their blood does not contain any of the A, B, or Rh antigens and can safely be given to anyone
AB positive: universal recipients because blood contains the A, B, and Rh antigens; they can safely receive any type of blood 23

24. Blood Product Transfusion
Transfusions of packed red blood cells
Consent signed before any blood transfusion
Blood sample drawn; sent for type and crossmatch
Policies for administering blood products vary; be familiar with and follow your institution’s policies
Autologous transfusion: using the patient’s own blood
Patient donates blood several times before procedure
Blood is stored by the blood bank and reinfused into the patient if needed intraoperatively or postoperatively
For each unit of packed red blood cells transfused, how much should the patient’s hemoglobin and hematocrit increase approximately?
One way to prevent the risks of infection and reactions with blood transfusions is to collect the patient’s own blood before a planned procedure and then transfuse the patient’s own blood back into the patient if needed as an autologous red blood cell transfusion. 24

25. Blood Product Transfusion
Platelet transfusion
When platelet count falls <20,000 cells/mm3
Patient must give signed consent
Sample sent to the blood bank for typing
Policies for administering blood products vary; be familiar with and follow your institution’s policies
Platelets are commonly ordered in four-packs or six-packs; each pack contains approximately 60 mL.
When should platelets be administered if they are being given to prevent bleeding during a procedure? 25

26. Blood Product Transfusion
Fresh frozen plasma transfusions
Plasma separated from whole blood by centrifugation and quickly frozen
Contains all the clotting factors as well as the plasma proteins
Cryoprecipitate contains only fibrinogen and factor VIII; can be further separated out from plasma and administered alone 26

27. Blood Product Transfusion
Reactions to blood transfusions
Four main types
Hemolytic
Anaphylactic
Febrile
Circulatory overload
Symptoms
Back or chest pain, fever, chills, decreased blood pressure, urticaria, wheezing, dyspnea, or coughing during the transfusion 27

28. Blood Product Transfusion
Reactions to blood transfusions
Interventions
Stop transfusion immediately; keep intravenous line open with normal saline
Immediately notify physician, nursing supervisor, blood bank
Be prepared to administer oxygen, epinephrine, Solu-Cortef, furosemide (Lasix), antipyretics as prescribed by physician
Save the unused portion of the blood bag for the blood bank
Be prepared to collect blood and urine samples from the patient for evaluation 28

29. Colony-Stimulating Factors
Naturally occurring hormones that stimulate the bone marrow to produce more blood cells
Erythropoietin (Epogen)
Stimulates bone marrow to produce more red blood cells
Effects on the hematocrit not apparent for several days; not an option for patients immediately needing to elevate their red blood cell counts
Erythropoietin is predominantly used for what type of patient? 29

30. Disorders of the Hematologic System: Red Blood Cell Disorders30

31. Polycythemia Vera Too many red blood cells are produced
Increased cells make blood more viscous (thicker); doesn’t circulate freely throughout body
Symptoms: headache, dizziness, ringing in the ears, and blurred vision. Patients with this disorder may have a ruddy (reddish) complexion
Treatment is to have a unit of blood phlebotomized, or taken off, to keep the hematocrit normal 31

32. Aplastic Anemia Complete failure of the bone marrow
Extremely low red blood cell count, white blood cell counts, and platelet counts because bone marrow is not making any of these cells
Certain drugs (e.g., streptomycin and chloramphenicol) and exposure to toxic chemicals/radiation cause bone marrow failure
In many cases, cause of bone marrow failure is never identified 32

33. Aplastic Anemia
Signs and symptoms: pallor, extreme fatigue, tachycardia, shortness of breath, hypotension, unusually prolonged or spontaneous bleeding, and frequent infections that do not resolve
Transfusion: replace red blood cells and platelets
Antibiotics to prevent or treat infections; corticosteroids also may be given
If bone marrow does not recover, a transplant may be considered if donor can be found 33

34. Autoimmune Hemolytic Anemia
Bone marrow makes enough blood cells, but they are destroyed once released into circulation
Causes: certain infections, drug reactions, and certain cancers
Hemolytic anemia of the newborn can occur after delivery if the mother has Rh-negative blood and the baby has Rh-positive blood
Transfusions can cause a hemolytic anemia if lymphocytes in the transfused blood make antibodies against the recipient 34

35. Autoimmune Hemolytic Anemia
Signs and symptoms: pallor, extreme fatigue, tachycardia, shortness of breath, and hypotension
Patients may appear jaundiced
High bilirubin levels from all the red blood cells lysed (broken down)
Patients have a positive Coombs’ antiglobulin blood test
Medical treatment: identifying and treating the cause
Blood transfusions may be needed to replace red blood cells
Corticosteroids may be administered to the patient
Patient usually recovers in a few days to weeks 35

36. Iron Deficiency Anemia
From a diet too low in iron or from the body not absorbing enough iron from the gastrointestinal tract
Symptoms: fatigue and pallor
Severe cases: orthostatic changes in heart rate and blood pressure
A low red blood cell count, low hemoglobin value, low hematocrit; a low serum iron level, a low ferritin level, and a high total iron-binding capacity
Physicians treat with ferrous sulfate and iron dextran
Encourage foods high in iron
Older adults with poor eating habits frequently suffer from anemia.
What foods are high in iron? 36

37. Pernicious Anemia (Vitamin B12 Anemia)
Vitamin B12 not absorbed from stomach
May lack intrinsic factor
May have had a gastrectomy
Symptoms: fatigue, pallor, weakness, a sore tongue, numbness of hands or feet
Treatment: monthly intramuscular injection of vitamin B12 (cyanocobalamin)
What nursing care is appropriate for patients receiving vitamin B12? 37

38. Sickle Cell Anemia Disk-shaped red blood cells become sickle shaped
Misshapen cells more fragile than normal red blood cells; as a result, the sickled cells easily rupture as they pass through small capillaries, resulting in a chronic anemia
Cells become stuck in the small capillaries, obstructing blood flow 38

39. Sickle Cell Anemia
Genetic disease: almost exclusively in African Americans
Carried on a recessive gene; a person must inherit the gene from both the mother and the father to actually have the disease
Sickle cell crisis: the sickled cells become stuck in larger blood vessels of the body; obstruct blood flow and cause severe pain
How many African Americans carry the genetic trait for sickle cell anemia?
Because sickle cell anemia is carried on a recessive gene, a person must inherit the gene from both the mother and the father to actually have the disease.
Newborn screening for hemoglobin S can be done to identify infants with sickle cell disease and to educate parents about the disease and prevention of crises. 39

40. Figure 32-4 40

41. Sickle Cell Anemia Various stressors can trigger a sickle cell crisis
Dehydration, infection, overexertion, cold weather changes, excessive alcohol consumption, smoking
Symptoms vary: depend on where circulation is blocked by the sickled red blood cells
Circulation to the chest, abdomen, bones, joints, bone marrow, brain, or penis may be compromised
Tissue hypoxia occurs, causing severe pain 41

42. Sickle Cell Anemia Medical diagnosis of sickle cell disease
Physicians use clinical judgment
Radiographs and scans of the painful area to evaluate for bleeding
Medical treatment of sickle cell crisis
There is no cure; treatment is symptomatic
Intravenous fluids and pain medication
Red blood cell transfusions correct the anemia and help the body oxygenate tissues
Oxygen therapy
Hydroxyurea
Aggressive intravenous hydration helps the kidneys clear the metabolic wastes from ruptured red blood cells.
Intravenous morphine commonly is prescribed for pain relief.
Will oxygen therapy help to reverse a sickle cell crisis?
Hydroxyurea stimulates the production of a certain type of hemoglobin that is resistant to sickling. 42

43. Sickle Cell Anemia Assessment Complete description of the pain
Document location, intensity, duration, and precipitating events; vital signs every 4 hours
Assess for fever
Any symptoms of an infection, such as sore throat, cough, abnormal breath sounds, dysuria, or diarrhea
Monitor for signs and symptoms of dehydration 43

44. Sickle Cell Anemia Interventions Acute Pain Anxiety Risk for Injury
Ineffective Therapeutic Regimen Management 44

45. Disorders of the Hematologic System: Coagulation Disorders45

46. Thrombocytopenia Too few platelets circulating in the blood
Not enough platelets being made in bone marrow or too many platelets are being destroyed in circulation
Major cause: treatment with chemotherapy or radiation therapy
Chemotherapy and radiation do not distinguish between rapidly dividing cancer cells and rapidly dividing normal cells like those that produce platelets.
When can thrombocytopenia be expected in a patient receiving chemotherapy or radiation? 46

47. Thrombocytopenia
Examples of too many platelets being either destroyed or consumed are idiopathic thrombocytopenic purpura (ITP) and thrombotic thrombocytopenic purpura (TTP)
Symptoms: petechiae and purpura, gingival bleeding, epistaxis (nosebleeds), or any other unusual or prolonged bleeding
Treatment for thrombocytopenia: treat or stop the causative factor 47

48. Disseminated Intravascular Coagulation
A hypercoagulable state
Overstimulation of normal coagulation cascade: simultaneous thrombosis and hemorrhage 48

49. Disseminated Intravascular Coagulation
Always secondary to another pathologic process: overwhelming sepsis, shock, major trauma, crush injuries, burns, cancer, acute tumor lysis syndrome, or obstetric complications (abruptio placentae, fetal demise)
Coagulation occurs at so many sites that eventually all available platelets and clotting factors are depleted and uncontrolled hemorrhage results 49

50. Disseminated Intravascular Coagulation
Blood tests that help diagnose DIC include prothrombin time, partial thromboplastin time, fibrinogen, thrombin time, fibrin split products level, and D-dimers
Blood component replacement therapy
Heparin to interrupt the DIC cycle and allow the body to replenish platelets and clotting factors
Some physicians believe additional platelets and clotting factors perpetuate the abnormal DIC feedback loop and so do not prescribe blood component replacement.
What nursing actions are appropriate for the patient who is at risk for injury from bleeding? 50

51. Hemophilia
Genetic disease: affected person lacks some blood clotting factors normally found in plasma
Signs and symptoms
Uncontrollable bleeding is the hallmark of hemophilia
Occurs after trauma; however, also spontaneously for no clear reason
Commonly, bleeding occurs into the joints, causing swelling and severe pain
Also can occur into the skin; from the mouth, gums, and lips; and from the gastrointestinal tract
What is the incidence of hemophilia?
In hemophilia A, factor VIII is missing, whereas in hemophilia B, factor IX is missing.
Regardless of whether the trait is inherited or spontaneous, it is rare for women to have this disease.
Because any surgical procedure puts the patient at great risk for bleeding, a complete preoperative evaluation must be done. 51

52. Hemophilia Medical diagnosis Medical treatment
Measuring factors VIII and IX in the blood
Partial thromboplastin time
Medical treatment
No cure; treatment is symptomatic
Physician prescribes transfusions of fresh frozen plasma or cryoprecipitate, or both
Red blood cell transfusions
Intravenous morphine
Physicians try quickly to transition from IV opioids to oral opioids to nonopioid pain relievers as crisis resolves 52

53. Hemophilia Assessment Interventions
For bleeding and pain; note what measures have stopped the bleeding and relieved pain in the past
Monitor vital signs and urine output
Interventions
Risk for Injury
Acute Pain
Ineffective Therapeutic Regimen Management 53