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Immunologic Disorders

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1 Immunologic Disorders
Chapter 33 Immunologic Disorders 1

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

3 Anatomy and Physiology of the Immune System
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4 Anatomic Structures and Components
Bone marrow Lymph, lymphatics, and lymph nodes Spleen Thymus Stem cells White blood cells (leukocytes) Neutrophils, monocytes and macrophages, eosinophils, basophils, mast cells, B lymphocytes (B cells), T lymphocytes (T cells) Cytokines Eicosanoids The bone marrow is the spongy center of the bones where the white blood cells are made. The lymphatic system is a network of open-ended tubes—separate from the blood circulation system—that collects the plasma left behind in the tissues and returns it to the venous system. The spleen filters microorganisms from the blood. Early in life certain white blood cells, called lymphocytes, migrate from the bone marrow where they are produced to the thymus where they mature into T lymphocytes, or T cells. Each type of white blood cell combats certain types of microorganisms. What does each type of WBC combat? Cytokines are hormones secreted by cells to signal other cells. Eicosanoids are a class of fatty acids that regulate blood vessel vasodilation, temperature elevation, white blood cell activation, and other physiologic processes involved in immunity. 4

5 Figure 33-1 5

6 Physiologic Functions: Innate Immunity
Operational at all times, whether or not a pathogen is present At birth, are immediately functional Include anatomic and physiologic barriers, inflammatory response, and the ability of certain cells to phagocytose foreign invaders 6

7 Physiologic Functions: Barriers
Skin and mucous membranes: a protective covering; secretes substances that inhibit pathogen growth Sweat glands secrete lysozyme, antimicrobial enzyme Acidic GI and GU systems inhibit growth of pathogenic organisms Secretions from the respiratory and gastrointestinal tracts contain the antibody IgA, as well as phagocytes Surfaces colonized by normal bacterial flora; prevents pathogens from attaching/gaining access to the body Coughing and sneezing, peristalsis in the GI tract, emptying the bladder, and sloughing of dead skin cells: remove microorganisms from the body, thus preventing their invasion and overgrowth 7

8 Physiologic Functions: Inflammation
Body initially responds to an injury or infection by dilating the capillary bed and increasing the capillary permeability of the affected area Brings white blood cells to the site; allows them to enter the tissue to attack microorganisms This multistep process is called inflammation and is recognized by rubor (redness), tumor (swelling), calor (heat), and dolor (pain) at the site of injury or infection 8

9 Physiologic Functions: Phagocytosis
The process of ingesting and digesting invading pathogens, dead cells, and cellular debris 9

10 Physiologic Functions: Acquired Immunity
Specific to particular pathogen: activated only when needed Antibody mediated Initiated when IgM immunoglobulins on B lymphocytes detect a foreign antigen Active: person synthesizes own antibodies in response to pathogen Passive: antibody produced by person or animal is transferred to another person When antibodies bind to an antigen, they do not actually destroy the pathogen, but they make the antigen readily recognizable to neutrophils, monocytes, and macrophages, which can phagocytose the pathogen. What is an example of active acquired immunity? Passive acquired immunity? 10

11 Physiologic Functions: Acquired Immunity
Cell mediated Aimed at intracellular defects caused by viruses and cancer Responsible for delayed hypersensitivity reactions and rejection of transplanted tissue 11

12 Figure 33-2 12

13 Physiologic Functions: Tolerance
Immune system must recognize its own proteins Autoimmune diseases occur when there is a breakdown of tolerance; immune system identifies its own proteins as foreign and mounts a response to destroy these self-proteins Examples: idiopathic thrombocytopenic purpura (ITP), thrombotic thrombocytopenic purpura (TTP), acute rheumatic fever, type 1 diabetes mellitus, systemic lupus erythematosus, rheumatoid arthritis, Graves’ disease, and Hashimoto’s thyroiditis 13

14 Age-Related Changes Bone marrow becomes less productive
Lymphatic tissue shrinks, resulting in fewer and smaller lymph nodes Immunologic function not affected unless a person is unusually stressed by trauma, a chronic infection, or treatment for cancer Immunologic function is generally not affected unless a person is unusually stressed by trauma, a chronic infection, or treatment for cancer. At what age does lymphatic tissue grow very quickly? 14

15 Nursing Assessment of the Immune System
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16 Chief Complaint and History of Present Illness
Frequent or persistent infections, bleeds for a long time when cut, bruises easily, or has chronic fatigue 16

17 Past Medical History Cancer or prior treatment for cancer, HIV infection, history of splenectomy, or an indwelling venous access device, indicating that the patient needed long-term venous access Medications patient is using or a recent change in medication may suggest an underlying immunologic problem Ask about recent changes in medications and recent immunizations 17

18 Family History Note family history of immunologic disorders such as cancer Death of a family member at a young age for reasons other than trauma may indicate a genetic immunologic disorder 18

19 Review of Systems Reddened, swollen, painful, warm areas
Ask about fever, chills, or night sweats Breaks in the skin, ulcers, lesions, or enlarged lymph nodes Assess for weakness, lethargy, malaise, restlessness, apprehension, or headache Ask about sinus pain, dyspnea, or cough Pain or burning with urination What is the primary symptom of an immunologic disorder? Night sweats can occur when the patient’s temperature rises at night but the patient does not awaken until the temperature falls, causing sweating. 19

20 Functional Assessment
Occupation and hobbies Self-concept Activity and exercise Sleep and rest Nutrition Interpersonal relationships Coping and stress Perception of health 20

21 Physical Examination General survey Skin
Responsiveness, mood, expression, posture Carefully inspect and describe reddened, swollen, or painful areas Skin Note general color, texture, turgor, temperature, and integrity of the skin Palpate for swollen or painful areas 21

22 Physical Examination Head and neck Thorax Lungs
Enlarged, swollen, or draining areas Thorax The examiner with advanced skills may palpate the axilla for enlarged lymph nodes Lungs Document respiratory rate and effort; auscultate for wheezing, crackles, or rhonchi 22

23 Physical Examination Heart and vascular system Abdomen
Heart rate and blood pressure Abdomen The examiner with advanced skills may palpate the abdomen for tenderness Palpate the groin for enlarged lymph nodes 23

24 Diagnostic Tests and Procedures
Blood tests Complete blood cell (CBC) count Antinuclear antibody test Enzyme-linked immunosorbent assay and Western blot tests Cultures of blood, urine, sputum, and stool Detect infections in blood, sputum, urine, stool Skin tests Physicians and nurses are primarily interested in the number of neutrophils because these are the cells that fight bacterial infections. What does a shift to the left or a shift to the right indicate on a CBC? Skin tests can serve as a barometer of immune system functioning, pointing out either hyposensitivities or hypersensitivities to a particular antigen. 24

25 Nursing Actions for the Patient at Risk for Injury from Infection
The lower a patient’s white blood cell count, in particular the lower the neutrophil count, the greater the patient’s risk of infection See Box 33-2, p. 599 25

26 Bone Marrow Transplant and Peripheral Blood Stem Cell Transplant
Restore hematologic and immunologic systems in patients with malignancies who have received extremely high doses of chemotherapy and radiation therapy Allogeneic bone marrow transplant Autologous bone marrow transplant Peripheral blood stem cell transplant Bone marrow transplantation is also used in patients with genetic bone marrow defects and aplastic anemia in an attempt to repopulate the bone marrow with blood-producing cells. How is a bone marrow transplant administered? 26

27 White Blood Cell Disorders of the Immune System: Neutropenia
Total number of neutrophils abnormally low, increasing risk of infection Caused by decreased bone marrow production, chemotherapy, radiation therapy, certain drugs or an autoimmune reaction Also by increased neutrophil utilization because of overwhelming infection Because these patients do not have adequate numbers of white blood cells to mount an immunologic response, classic signs of infection (redness, swelling, and pain) may be absent. What may be the only sign of infection in a patient who has had neutropenia for a long period of time? 27

28 White Blood Cell Disorders of the Immune System: Neutropenia
Common sites of infection: lung, blood, skin, urinary tract, gastrointestinal tract Goal of antibiotic therapy: support patient until patient’s own white blood cells are available to fight the infection Important to minimize patient’s exposure to infectious agents by instituting compromised host precautions Usually bacteria cause infections in neutropenic patients; however, fungi and viruses can also infect these patients. What precautions should be taken when a patient is placed on compromised host precautions? 28

29 Leukemia Cancer of the white blood cells: bone marrow produces too many immature white blood cells Immature white blood cells leave patient at great risk for life-threatening infections Factors: exposure to large doses of ionizing radiation or exposure to certain chemicals such as benzene, a compound found in gasoline Two main types—myelogenous and lymphocytic Each type can be either chronic or acute The American Cancer Society statistics estimated that 30,800 cases of leukemia would be diagnosed in 2002, accounting for 2% of all new cases of cancer diagnosed in that year. Which types of leukemia most commonly occur in adults? 29

30 Leukemia Signs and symptoms of acute leukemia Fevers and night sweats
Fatigue, paleness, tachycardia, and tachypnea Petechiae or purpura, epistaxis, gingival bleeding, melena, or menorrhagia Weight loss and swollen lymph nodes Concurrently, patients may have symptoms related to low platelet counts, such as petechiae or purpura, epistaxis (nose bleeds), gingival bleeding (from the gums), melena (blood in the stool), or menorrhagia (heavy menstrual bleeding). What may cause bone pain to occur in a patient with leukemia? 30

31 Leukemia Medical diagnosis of acute leukemia
CBC with extremely high white blood cell count Bone marrow biopsy Medical treatment of acute leukemia Chemotherapy Antibiotics and blood transfusions Acute leukemia is initially treated using high doses of chemotherapy to destroy the diseased bone marrow and allow the body to regrow healthy bone marrow. What is an example of intensification therapy? 31

32 Leukemia Assessment Signs or symptoms of infection
Complete vital signs every 4 hours If sputum, note the amount and color Skin for reddened, swollen, painful, or draining areas Inspect the mouth and pharynx for reddened, swollen, painful, or draining areas Assess for any evidence of bleeding Petechiae, purpura, or ecchymoses Perform a guaiac test Fever is the hallmark of infection. Why is it possible that a patient with leukemia may have an infection with no production of pus? Also note any side effects from the chemotherapy itself, such as nausea and vomiting or stomatitis, so the appropriate interventions can be taken. 32

33 Leukemia Interventions Risk for Injury Fatigue
Impaired Oral Mucous Membranes Imbalanced Nutrition: Less Than Body Requirements Anxiety Ineffective Therapeutic Regimen Management 33

34 Hypersensitivity Reactions
Exaggerated immune responses that can be uncomfortable and harmful to the patient Four types of hypersensitivity reactions, classified according to the time between exposure and reaction, immune mechanism involved, and site of reaction 34

35 Hypersensitivity Reactions
Type I Immediate hypersensitivity reactions are mediated by IgE reacting to common allergens, such as dust, pollen, animal dander, insect stings, or various drugs Either local, resulting in local swelling and discomfort, or systemic, resulting in anaphylaxis and possible death if not recognized and treated promptly 35

36 Hypersensitivity Reactions
Type II Immediate hypersensitivity reactions are mediated by antibody reactions Can occur with a mismatched blood transfusion or as response to various drugs 36

37 Hypersensitivity Reactions
Type III Immediate hypersensitivity reactions result in tissue damage resulting from precipitation of antigen-antibody immune complexes Can occur with autoimmune reactions, some occupational diseases, or as response to various drugs 37

38 Hypersensitivity Reactions
Type IV Delayed hypersensitivity reactions result from immune cells migrating to the site of exposure days after the exposure to the antigen Can occur with contact dermatitis, measles rash, tuberculin skin testing, or various drugs 38

39 Idiopathic Thrombocytopenic Purpura (ITP)
IgG mistakenly helps destroy patient’s platelets Drugs that induce ITP: sulfonamides, thiazide diuretics, chlorpropamide, quinidine, and gold. Patients with HIV are at increased risk for developing ITP Treatment: steroids and intravenous immune globulin (IVIG) Immunosuppressive therapy with cytotoxic drugs (e.g., vincristine or cyclophosphamide) can be used in patients who do not respond to splenectomy. Why is a platelet transfusion not indicated with ITP? 39

40 Thrombotic Thrombocytopenic Purpura (TTP)
Exaggerated immunologic response to vessel injury that results in extensive clot formation and decreased blood flow to the site Patients critically ill; develop fever, thrombocytopenia, hemolytic anemia, renal impairment, and neurologic symptoms The main treatment is plasmapheresis Others: steroids, antiplatelet agents (e.g., aspirin, dipyridamole, Persantine), splenectomy, or all three Plasmapheresis presumably removes the immunologic agent that triggered the TTP from the plasma. Why are platelet transfusions contraindicated with TTP? 40

41 Systemic Lupus Erythematosus
Autoimmune disease: immune system unable to recognize itself; mounts an immune response against its own proteins Damage from antibodies and immune complexes directed against one/many organs The cause is unknown Cause of death: infection and disease of the cardiovascular, renal, pulmonary, and central nervous systems Ninety percent of cases occur in women, usually of childbearing age. In which ethnic groups is SLE most common? Factors associated with a poor outcome include increased creatinine, hypertension, large amounts of protein excreted in the urine, anemia, and low socioeconomic status. 41

42 Systemic Lupus Erythematosus
Signs and symptoms Fatigue, malaise, fever, anorexia, nausea, and weight loss Arthralgias and myalgias Joints often swollen, tender, stiff, and painful Rash and photosensitivity Butterfly-shaped rash across the bridge of the nose and the cheeks Inflammation of the retina can result in sudden-onset blindness 42

43 Systemic Lupus Erythematosus
Diagnosis No one test confirms the diagnosis of SLE Based on constellation of symptoms If any four of the following symptoms: SLE Characteristic rash Photosensitivity with exposure to sunlight Oral ulcers Arthritis Pleuritis or pericarditis Renal, neurologic, hematologic, or immunologic disorder Positive ANA 43

44 Systemic Lupus Erythematosus
Medical treatment There is no cure Treatment: symptomatic and aimed at minimizing symptoms, preventing organ damage, and maintaining quality of life Analgesics, nonsteroidal anti-inflammatory drugs (NSAIDs), antimalarials, corticosteroids Cytotoxic agents suppress the abnormal immune response 44

45 Systemic Lupus Erythematosus
Assessment Thorough health history and physical examination Complete functional assessment: determine the effects of the symptoms on the activities of daily living 45

46 Systemic Lupus Erythematosus
Interventions Fatigue Acute Pain Disturbed Body Image Ineffective Coping Ineffective Therapeutic Regimen Management 46

47 Human Immunodeficiency Virus (HIV)
A retrovirus that infects cells expressing CD4 on their cell membranes, primarily TH cells 47

48 Transplant Rejection Patients who undergo kidney, heart, liver, or other organ transplantation risk that their healthy immune system will recognize the transplanted organ as foreign and try to destroy it 48

49 Transplant Rejection Rejections occur because
T lymphocytes can directly attack the allograft, resulting in acute transplant rejection within hours of the transplant B lymphocytes can make antibodies against the allograft Fibrin accumulates on the transplanted tissue, causing ischemia 49

50 Transplant Rejection Donor-recipient tissue matching minimizes recipient’s immune system attacking allograft Drug combinations suppress recipient’s immune system and minimize the immune response to the allograft Drugs suppress the patient’s ability to fight bacteria, viruses, fungi, and parasites, putting at risk for infection Combinations of the steroids cyclosporine and azathioprine chronically suppress the immune system after an organ transplant 50

51 Transplant Rejection Several newer drugs target the T cells while preserving B cell function and thus more of the patient’s immune function Tacrolimus (Prograf), antilymphocyte globulin, antithymocyte globulin, and murine monoclonal antibody to CD3 (OKT3) Patients who have undergone organ transplantation must take immunosuppressive therapy the rest of their lives to preserve the allograft 51


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