IMMUNOLOGIC DISORDERS




Acquired Immune Deficiency Syndrome (AIDS)

  1. General information
    1. Characterized by severe deficits in cellular immune function; manifested clinically by opportunistic infections and/or unusual neoplasms
    2. Etiologic factors
      1. Results from infection with human immunodeficiency virus (HIV), a retrovirus that preferentially infects helper T-lymphocytes (T4 cells)
      2. Transmissible through sexual contact, contaminated blood or blood products, and from infected woman to child in utero or possibly through breast-feeding
      3. HIV is present in an infected person's blood, semen, and other body fluids
    3. Epidemiology is similar to that of hepatitis B; increased incidence in populations in which sexual promiscuity is common and in IV drug abusers
  2. Medical management
    1. No effective cure for AIDS at present; several categories of antiretroviral drugs now available
      1. Nucleoside Analogues: Didanosine (Videx) (ddl), Lamivudine (3TC) (Epivir), Stavudine (d4T) (Zerit), Zidovudine (AZT) (Retrovir)
      2. Nucleoside Analogues: Didanosine (Videx) (ddl), Lamivudine (3TC) (Epivir), Stavudine (d4T) (Zerit), Zidovudine (AZT) (Retrovir)
      3. Non Nucleoside Analogues: Delavirdine (DLV) (Rescriptor), Nevirapine (NVP) (Viramune)
      4. Protease Inhibitors: Indinavir (Crixivan), Nelfinavir (Viracept), Ritonavir (Norvir), Saquinavir (Invirase)
    2. Primary goal of treatment is to treat opportunistic infections and cancers that develop and provide supportive care for the effects of the disease, e.g., diarrhea, malnutrition, mental changes, etc.
    3. Drugs used to treat PCP include
      1. PO or IV trimethoprim-sulfamethoxazole (Bactrim, Septra); side effects include rash, leukopenia, fever
      2. IM or IV pentamidine (Pentam 300); side effects include hepatotoxicity, nephrotoxicity, blood sugar imbalances, abscess or necroses at IM injection site, hypotension
  3. Assessment findings (see Table 4.18)
    1. Fatigue, weakness, anorexia, weight loss, diarrhea, pallor, fever, night sweats
    2. Shortness of breath, dyspnea, cough, chest pain, and progressive hypoxemia secondary to infection (pneumonia)
    3. Progressive weight loss secondary to anorexia, nausea, vomiting, diarrhea, and a general wasting syndrome; fatigue, malaise
    4. Temperature elevations (persistent or intermittent); night sweats
    5. Neurologic dysfunction secondary to acute meningitis, progressive dementia, encephalopathy, encephalitis
    6. Presence of opportunistic infection, for example
      1. Pneumocystis carinii pneumonia
      2. Herpes simplex, cytomegalovirus, and Epstein-Barr viruses
      3. Candidiasis: oral or esophageal
      4. Mycobacterium-avium complex
    7. Neoplasms
      1. Kaposi's sarcoma
      2. CNS lymphoma
      3. Burkitt's lymphoma
      4. Diffuse undifferentiated non-Hodgkin's lymphoma
    8. Laboratory findings: diagnosis based on clinical criteria and positive HIV antibody test--ELISA (enzyme-linked immunosorbent assay) confirmed by Western blot assay. Other lab findings may include
      1. Leukopenia with profound lymphopenia
      2. Anemia
      3. Thrombocytopenia
      4. Decreased circulatory T4 lymphocyte cells
      5. Low T4:T8 lymphocyte ratio
  4. Nursing interventions
    1. Administer medications as ordered for concomitant disease; monitor for signs of medication toxicity.
    2. Monitor respiratory status; provide care as appropriate for respiratory problems, e.g., pneumonia.
    3. Assess neurological status; reorient client as needed; provide safety measures for the confused/disoriented client.
    4. Assess for signs and symptoms of fluid and electrolyte imbalances; monitor lab studies; ensure adequate hydration.
    5. Monitor client's nutritional intake; provide supplements, total parenteral nutrition, etc., as ordered.
    6. Assess skin daily (especially perianal area) for signs of breakdown; keep skin clean and dry; turn q4 hours while in bed.
    7. Inspect oral cavity daily for ulcerations, signs of infection; instruct client to rinse mouth with normal saline and hydrogen peroxide or normal saline and sodium bicarbonate rinses.
    8. Observe for signs and symptoms of infection; report immediately if any occur.
    9. If severe leukopenia develops, institute neutropenic precautions
      1. Prevent trauma to skin and mucous membranes, e.g., avoid enemas, rectal temperatures; minimize all parenteral infections
      2. Do not place client in a room with clients having infections
      3. Screen visitors for colds, infections, etc.
      4. Do not allow fresh fruits, vegetables, or plants in client's room.
      5. Mask client when leaving room for walks, x-rays, etc.
    10. Institute blood and body fluid precautions (see Nursing Responsibilities in Prevention of Spread of Infection)
    11. Provide emotional support for client/significant others; help to decrease sense of isolation
    12. Provide client teaching and discharge planning concerning
      1. Importance of observing for signs of infections and notifying physician immediately if any occur
      2. Ways to reduce chance of infection
        1. Clean kitchen and bathroom surfaces regularly with disinfectants.
        2. Avoid direct contact with pet's litter boxes or stool, bird cage droppings, and water in fish tanks.
        3. Avoid contact with people with infections, e.g., cold, flu.
        4. Importance of balancing activity with rest.
        5. Need to eat a well-balanced diet with plenty of fluids.
      3. Prevention of disease transmission
        1. Use safer sex practices, e.g., condoms for sexual intercourse.
        2. Do not donate blood, semen, organs.
        3. Do not share razors, toothbrushes, or other items that may draw blood.
        4. Inform all physicians, dentists, sexual partners of diagnosis.
      4. Resources include Public Health Service, National Gay Task Force, American Red Cross, local support groups



TABLE 4.18 Classification System for HIV Infection


CD4 + T-cell categories

A
Asymptomatic, acute HIV or PGL

B
Symptomatic, not (A) or (C) conditions

C
AIDS-indicator conditions

(1) 500/uL

A1

B1

C1

(2) 200-499/uL

A2

B2

C2

(3) <200/ul

A3

B3

C3

Clinical Category A

Clinical Category B

Clinical Category C

1 or more of the following, confirmed HIV infection, and without conditions in B and C
* Asymptomatic HIV infection
* Persistent Generalized Lymphadenopathy (PGL)
* Acute (primary) HIV infection with accompanying illness or history of acute HIV infection

* Candidiasis (oral or vaginal), frequent or poorly resistant to therapy
* Cervical dysplasia/cervical carcinoma in situ
* Fever or diarrhea exceeding 1 month
* Hairy leukoplakia, oral
* Herpes zoster, involving 2 episodes or more than one dermatome
* ITP
* PID
* Peripheral neuropathy

* Candidiasis of bronchi, trachea, or lungs
* Cervical cancer, invasive
* Coccidiomycosis
* Cryptosporidiosis
* Cytomegalovirus
* Encephalopathy
* Herpes simplex: chronic ulcer - exceeding 1 month duration
* Histoplasmosis
* Kaposi's sarcoma
* Lymphoma
* Mycobacterium - avium complex
* Mycobacterium tuberculosis
* Pneumocystis carinii pneumonia
* Salmonella
* Toxoplasmosis of brain
* Wasting syndrome due to HIV

Adopted from Centers for Disease Control, U.S. Dept. of Health and Human Services, 1993 revised classification system for HIV infections and expanded surveilance case definition for AIDS among adolescents and adults.




Malignancies


Multiple Myeloma

  1. General information
    1. A neoplastic condition characterized by the abnormal proliferation of plasma cells in the bone marrow, causing the development of single or multiple tumors composed of abnormal plasma cells. Disease disseminates into lymph nodes, liver, spleen, and kidneys and causes bone destruction throughout the body.
    2. Cause unknown, but environmental factors thought to be involved
    3. Disease occurs after age 40; affects men twice as often as women
    4. Pathophysiology
      1. Bone demineralization and destruction with osteoporosis and a negative calcium balance
      2. Disruption of erythrocyte, leukocyte, and thrombocyte production
  2. Medical management
    1. Drug therapy
      1. Analgesics for bone pain
      2. Chemotherapy (melphalan [Alkeran] and cyclophosphamide [Cytoxan]) to reduce tumor mass; may intensify the pancytopenia to which these clients are prone; requires careful monitoring of laboratory studies
      3. Antibiotics to treat infections
      4. Gammaglobulin for infection prophylaxis
      5. Corticosteroids and mithramycin for severe hypercalcemia
    2. Radiation therapy to reduce tumor mass and for palliation of bone pain
    3. Transfusion therapy
  3. Assessment findings
    1. Headache and bone pain increasing with activity
    2. Pathologic fractures
    3. Skeletal deformities of sternum and ribs
    4. Loss of height (spinal column shortening)
    5. Osteoporosis
    6. Renal calculi
    7. Anemia, hemorrhagic tendencies, and increased susceptibility to infection
    8. Hypercalcemia
    9. Renal dysfunction secondary to obstruction of convoluted tubules by coagulated protein particles
    10. Neurologic dysfunction: spinal cord compression and paraplegia
    11. Laboratory tests
      1. Radiologic: diffuse bone lesions, widespread demineralization, osteoporosis, osteolytic lesions of skull
      2. Bone marrow; many immature plasma cells; depletion of other cell types
      3. CBC: reduced Hgb, WBC, and platelet counts
      4. Serum globulins elevated
      5. Bence-Jones protein: positive (abnormal globulin that appears in the urine of clients with multiple myeloma and other bone tumors)
  4. Nursing interventions
    1. Provide comfort measures to help alleviate bone pain.
    2. Encourage ambulation to slow demineralization process.
    3. Promote safety as clients are prone to pathologic and other fractures.
    4. Encourage fluids: 3000-4000 ml/day to counteract calcium overload and to prevent protein from precipitating in the renal tubules.
    5. Provide nursing care for clients with bleeding tendencies and susceptibility to infection.
    6. Provide a supportive atmosphere to enhance communication and reduce anxiety.
    7. Provide client teaching and discharge planning concerning
      1. Crucial importance of long-term hydration to prevent urolithiasis and renal obstruction
      2. Safety measures vital to decrease the risk of injury
      3. Avoidance of crowds or sources of infection if leukopenic


Polycythemia Vera

  1. General information
    1. An increase in both the number of circulating erythrocytes and the concentration of Hgb within the blood
    2. Three forms: polycythemia vera, secondary polycythemia, and relative polycythemia
    3. Classified as a myeloproliferative disorder (bone marrow overgrowth)
    4. Cause unknown, but thought to be a form of malignancy similar to leukemia
    5. Usually develops in middle age, common in Jewish men
    6. Pathophysiology
      1. A pronounced increase in the production of erythrocytes accompanied by an increase in the production of myelocytes (leukocytes within bone marrow) and thrombocytes.
      2. The consequences of this overproduction are an increase in blood viscosity, an increase in total blood volume (2-3 times greater than normal), and severe congestion of all tissues and organs with blood.
  2. Assessment findings
    1. Ruddy complexion and duskiness of mucosa secondary to capillary congestion in the skin and mucous membranes
    2. Hypertension associated with vertigo, headache, and "fullness" in the head secondary to increased blood volume
    3. Symptoms of CHF secondary to overwork of the heart
    4. Thrombus formation: CVA, MI, gangrene of the extremities, DVT, and pulmonary embolism can occur
    5. Bleeding and hemorrhage secondary to congestion and overdistension of capillaries and venules
    6. Hepatomegaly and splenomegaly
    7. Peptic ulcer secondary to increased gastric secretions
    8. Gout secondary to increased uric acid released by nucleoprotein breakdown
    9. Laboratory tests
      1. CBC: increase in all mature cell forms (erythrocytes, leukocytes, and platelets)
      2. Hct: increased
      3. Bone marrow: increase in immature cell forms
      4. Bilirubin (indirect): increase in unconjugated fraction
      5. Liver enzymes may be increased
      6. Uric acid increased
      7. Hematuria and melena possible
  3. Nursing interventions
    1. Monitor for signs and symptoms of bleeding complications.
    2. Force fluids and record I&O.
    3. Prevent development of DVT.
    4. Monitor for signs and symptoms of CHF.
    5. Provide care for the client having a phlebotomy.
    6. Prevent/provide care for bleeding or infection complications.
    7. Administer medications as ordered.
      1. Radioactive phosphorus (32P): reduction of erythrocyte production, produces a remission of 6 months to 2 years
      2. Nitrogen mustard, busulfan (Myleran), chlorambucil, cyclophosphamide to effect myelosuppression
      3. Antigout and peptic ulcer drugs as needed.
    8. Provide client teaching and discharge planning concerning
      1. Decrease in activity tolerance, need to space activity with periods of rest
      2. Phlebotomy regimens: outpatient frequency is determined by hct; importance of long-term therapy
      3. High fluid intake
      4. Avoidance of iron-rich foods to avoid counteracting the therapeutic effects of phlebotomy
      5. Recognition and reporting of bleeding
      6. Need to avoid persons with infections, especially in leukopenic clients.



Leukemia

  1. General information
    1. Most common form of childhood cancer
    2. Peak incidence is 3 to 5 years of age
    3. Proliferation of abnormal white blood cells that do not mature beyond the blast phase
    4. In the bone marrow, blast cells crowd out healthy white blood cells, red blood cells, and platelets, leading to bone marrow depression
    5. Blast cells also infiltrate other organs, most commonly the liver, spleen, kidneys, and lymph tissue
    6. Symptoms reflect bone marrow failure and associated involvement of other organs
    7. Types of leukemia, based on course of disease and cell morphology
      1. Acute lymphocytic leukemia (ALL)
        1. 80-85% of childhood leukemia
        2. malignant change in the lymphocyte or its precursors
        3. acute onset
        4. 95% chance of obtaining remission with treatment
        5. 75% chance of surviving 5 years or more
        6. prognostic indicators include: initial white blood count (less than 10,000/mm3), child's age (2-9 years), histologic type, sex
      2. Acute nonlymphocytic leukemia (ANLL)
        1. includes granulocytic and monocytic types
        2. 60-80% will obtain remission with treatment
        3. 30-40% cure rate
        4. prognostic indicators less clearly defined
  2. Medical management
    1. Diagnosis: blood studies, bone marrow biopsy
    2. Treatment stages
      1. Induction: intense and potentially life threatening
      2. CNS prophylaxis: to prevent central nervous system disease. Combination of radiation and intrathecal chemotherapy.
      3. Maintenance: chemotherapy for 2 to 3 years.
  3. Assessment findings
    1. Anemia (due to decreased production of RBCs), weakness, pallor, dyspnea
    2. Bleeding (due to decreased platelet production), petechiae, spontaneous bleeding, ecchymoses
    3. Infection (due to decreased WBC production), fever, malaise
    4. Enlarged lymph nodes
    5. Enlarged spleen and liver
    6. Abdominal pain with weight loss and anorexia
    7. Bone pain due to expansion of marrow
  4. Nursing interventions
    1. Provide care for the child receiving chemotherapy and radiation therapy.
    2. Provide support for child/family; needs will change as treatment progresses.
    3. Support child during painful procedures (frequent bone marrow aspirations, lumbar punctures, venipunctures needed).
      1. Use distraction, guided imagery.
      2. Allow child to retain as much control as possible.
      3. Administer sedation prior to procedure as ordered.



Hodgkin's and Non-Hodgkin's Lymphoma

Hodgkin's Lymphoma

  1. General information
    1. Malignant neoplasm of lymphoid tissue, usually originating in localized group of lymph nodes; a proliferation of lymphocytes
    2. Metastasizes first to adjacent lymph nodes
    3. Cause unknown
    4. Most prevalent in adolescents; accounts for 5% of all malignancies
    5. Prognosis now greatly improved for these children; influenced by stage of disease and histologic type
    6. Long-term treatment effects include increased incidence of second malignancies, especially leukemia and infertility
  2. Medical management
    1. Diagnosis: extensive testing to determine stage, which dictates treatment modality
      1. Lymphangiogram determines involvement of all lymph nodes (reliable in 90% of clients); is helpful in determining radiation fields
      2. Staging via laparotomy and biopsy
        1. stage I: single lymph node involved; usually in neck; 90%-98% survival
        2. stage II: involvement of 2 or more lymph nodes on same side of diaphragm; 70%-80% survival
        3. stage III: involvement of nodes on both sides of diaphragm; 50% survival
        4. stage IV: metastasis to other organs
      3. Laparotomy and splenectomy
      4. Lymph node biopsy to identify presence of Reed-Sternberg cells and for histologic classification
    2. Radiation: used alone for localized disease
    3. Chemotherapy: used in conjunction with radiation therapy for advanced disease
  3. Assessment findings
    1. Major presenting symptom is enlarged nodes in lower cervical region; nodes are nontender, firm, and movable
    2. Recurrent, intermittent fever
    3. Night sweats
    4. Weight loss, malaise, lethargy
    5. Pruritus
    6. Diagnostic test: presence of Reed-Sternberg cells
  4. Nursing interventions
    1. Provide care for child receiving radiation therapy.
    2. Administer chemotherapy as ordered and monitor/alleviate side effects.
    3. Protect client from infection, especially if splenectomy performed.
    4. Provide support for child/parents; specific needs of adolescent client must be considered.


Non-Hodgkin's Lymphoma

  1. General information
    1. Tumor originating in lymphatic tissue
    2. Significantly different from Hodgkin's lymphoma
      1. Control of primary tumor is difficult
      2. Disease is diffuse, cell type undifferentiated
      3. Tumor disseminates early
      4. Includes wide range of disease entities: lymphosarcoma, reticulum cell sarcoma, Burkitt's lymphoma
    3. Primary sites include GI tract, ovaries, testes, bone, CNS, liver, breast, subcutaneous tissues
    4. Affects all age groups.
  2. Medical management
    1. Chemotherapy: multiagent regimens including cyclophosphamide (Cytoxan), vincristine, prednisone, procarbazine, doxorubicin, bleomycin
    2. Radiation therapy: primary treatment in localized disease
    3. Surgery for diagnosis and clinical staging
  3. Assessment findings
    1. Depend on anatomic site and extent of involvement
    2. Rapid onset and progression
    3. Many have advanced disease at diagnosis
  4. Nursing interventions: provide care for child receiving chemotherapy, radiation therapy, and surgery.

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