Chapter 32

1. Chapter 32 Hematologic Disorders

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 System

4. Structures and Components of the Hematologic System Bone marrow Liver Spleen Blood Red blood cells (erythrocytes) Platelets (thrombocytes) Clotting factors Plasma

5. Figure 32-1

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

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)

8. Figure 32-2

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

10. Nursing Assessment of the Hematologic System

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

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

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

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

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

16. Functional Assessment Occupation and hobbies Self-concept Activity and exercise Sleep and rest Interpersonal relationships Coping and stress Perception of health

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

18. Physical Examination Thorax Lungs 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

19. Diagnostic Tests and Procedures Blood tests Red blood cell count Hemoglobin (Hb or Hgb) Hematocrit (Hct) Platelet counts Bone marrow biopsy

20. Common Therapeutic Measures

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

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

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

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

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

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

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

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

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

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

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

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

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

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-positiveblood Transfusions can cause a hemolytic anemia if lymphocytes in the transfused blood make antibodies against the recipient

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

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

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)

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

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

40. Figure 32-4

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

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

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

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

45. Disorders of the Hematologic System: Coagulation Disorders

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

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

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

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

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