Chapter 36 Hodgkin’s Disease
PATHOPHYSIOLOGY
Hodgkin’s disease is a malignancy of the lymphoid system. The cause remains unknown. Hodgkin’s disease is seen with a higher frequency in children with inherited immune deficiency syndromes. There has also been an association for some patients following infection with Epstein-Barr virus. Hodgkin’s disease is characterized by a proliferation of Reed-Sternberg cells that are surrounded by a pleomorphic infiltrate of reactive cells. Although no tissue is exempt from involvement, Hodgkin’s disease primarily affects nodal or lymphatic sites. The liver, the spleen, the bone marrow, and the lungs may also be involved. Hodgkin’s disease is classified according to the predominating type of cells and is characterized by four histologic states: (1) lymphocytic predominance (rare in children, often very localized disease requiring minimal therapy), (2) nodular sclerosis (most common in children), (3) mixed cellularity, and (4) lymphocytic depletion.
High survival rates have been achieved in children with Hodgkin’s disease as a result of improved staging procedures, combined therapies, and advances in supportive care. The 5-year relative survival rate for children under age 15 has gone from 78% in 1976 to 95% in 2001. However, cure often comes with significant cost secondary to the late effects of therapy. Over the past 2 decades, there has been a focus on lowering the doses and fields of radiation and limiting the exposure of certain chemotherapeutic agents. Newer treatment protocols are also evaluating therapy that is based on the individual’s response to initial treatment. If there is an early complete response to treatment, then the remaining therapy is less intense. The hope is that cure rates can be maintained with fewer long-term side effects. Early results have been encouraging.
INCIDENCE
1. Hodgkin’s disease accounts for 5% of malignancies in children in the United States.
2. Hodgkin’s disease has been reported in infants and young children but is rare before the age of 5 years.
3. Only 10% to 15% of cases occur in children younger than 16 years of age. The majority of cases in children occur in children aged 11 years and older.
4. Before age 10 year, Hodgkin’s disease is more common in males. After age 10, the ratio approaches 1:1.
5. The 5-year survival rate for stages I and II disease is 85% to 95%; for stages III and IV disease, 70% to 90%.
CLINICAL MANIFESTATIONS
1. Lymphadenopathy, usually in the cervical, supraclavicular, and mediastinal areas; mediastinal presentation common in adolescents and young adults; significant mediastinal adenopathy may cause cough, dyspnea, or superior vena cava syndrome
2. Painless, movable lymph nodes in tissues surrounding involved area
COMPLICATIONS
Common Acute Complications of Therapy
1. Bone marrow suppression—puts patient at risk for infection, bleeding, fatigue
2. Hair loss—very likely with all Hodgkin’s therapy regimens for children
3. Nausea and vomiting—often can be prevented or minimized with newer and combination antiemetic regimens
4. Constipation—likely with regimens that include oncovin(vincristine); associated with decreased mobility and use of narcotic pain medications
5. Diarrhea—may occur after prolonged antibiotic use; may be secondary to infection such as Clostridium difficile
6. Mucositis—secondary to mucosal damage from chemotherapy or radiation—may be accompanied by fungal infection (oral candidiasis)
7. Weight loss—secondary to anorexia, altered taste
8. Pain—after procedures such as central line placement or bone marrow or node biopsy
9. Allergic or anaphylactic reactions—may occur with some chemotherapy agents such as bleomycin, or with blood products
10. Gastritis—secondary to steroids administration, stress, vomiting
11. Mood swings, fluid retention, and altered glucose metabolism—secondary to steroids administration
12. Anxiety, behavioral problems such as regression, sleep disturbances—secondary to diagnosis and necessary treatments and procedures
Potential Late Complications Related to Treatment
1. Endocrine dysfunction including hypothyroidism, premature ovarian failure, sterility in males, and osteopenia
2. Cardiac dysfunction, including pericardial effusion, valvular heart disease, coronary artery disease, and constrictive pericarditis with tamponade
3. Pulmonary dysfunction, including fibrosis or pneumonitis
4. Impaired immunity (during therapy and in the first year posttherapy)
5. Soft tissue and bone growth impairment in irradiated fields
6. Secondary malignancy, including thyroid carcinoma, basal cell carcinoma, osteosarcomas, breast and colon carcinomas, soft tissue sarcomas, non-Hodgkin’s lymphoma, and leukemia
LABORATORY AND DIAGNOSTIC TESTS
1. Complete blood count—diagnostic (anemia may indicate advanced disease)
2. Erythrocyte sedimentation rate (ESR)—may be elevated at diagnosis
3. Serum copper, iron, calcium, and alkaline phosphatase levels—also may be elevated at diagnosis
4. Liver and renal function tests—to assess organ involvement
5. Urinalysis—to determine renal involvement
6. Chest radiographic study—to determine mediastinal or hilar node involvement
7. Computed tomography—to evaluate mediastinal, pulmonary, and abdominal disease
8. Gallium and/or positron emission tomography (PET) scan to determine the extent of involvement
9. Excisional lymph node biopsy—essential to diagnosis and staging
10. Bone marrow biopsy if patient has stage 3 or 4 disease according to imaging studies
MEDICAL MANAGEMENT
Staging is used to determine the anatomic extent of the disease at the time of diagnosis and to select the most appropriate therapy (Box 36-1). Staging includes biopsy, history, physical examination, and radiographic data collection. With advances in radiographic techniques, a staging laparotomy that formerly would have been done is not routinely recommended. Even with the best radiographic techniques, false positives and false negatives occur. Also, because all children and adolescents on treatment protocols receive chemotherapy now (and not just local radiation), staging laparotomy is no longer routinely performed.
| Stage | Description* |
|---|---|
| I | Involvement of a single lymph node region or a single extralymphatic organ or site |
| II | Involvement of two or more lymph node regionson the same side of the diaphragm or localized involvement of an extralymphatic organ or site and one or more lymph node regions on the same side of the diaphragm |
| III | Involvement of lymph node regions or extralymphatic organs or sites or spleen on both sides of the diaphragm |
| IV | Diffuse involvement of one or more extralymphatic organs or tissues with or without associated lymph node involvement |
* Subdivision A has no defined symptoms; subdivision B symptoms include unexplained recent weight loss or fever or night sweats.
At the time of diagnosis, approximately 60% of children with Hodgkin’s disease have stage I or II disease. Stage III disease is diagnosed in approximately 30% of children, and 10% have stage IV disease. Approximately 30% of children have B symptoms (unexplained fever, more than 10% weight loss, night sweats). Those with B symptoms are treated more intensively. The absence of these B symptoms is known as A classification. The treatment approach is guided by the stage of the disease at diagnosis. The goal of treatment is cure of the disease with minimal treatment-related toxicities and sequelae.
Over the last 30 years, the treatment for Hodgkin’s disease has evolved significantly. In the 1960s, radiation was the mainstay of therapy. Though this was curative for localized disease, those with advanced disease were rarely cured. In the 1960s a drug combination known as MOPP (nitrogen mustard, oncovin, prednisone, procarbazine) was used for those with advanced disease. In combination with radiation, more patients with advanced disease were cured. However, these patients often suffered significant side effects such as infertility, secondary leukemia, and other secondary malignancies. In the 1970s, a new drug combination known as ABVD (adriamycin, bleomycin, velban, and dacarbazine) was developed. This regimen was determined to be effective, with fewer problematic side effects than MOPP. In the 1980s, these two drug combinations were alternated to minimize the dose-related side effects of each regimen. Protocols were subsequently developed using chemotherapy with reduced radiation doses and fields. Today, the treatment for Hodgkin’s disease continues to evolve and is now risk-adapted. The most intensive regimens are used only for patients with advanced disease. Several new drug combinations are being used: BEACOPP (bleomycin, etoposide, adriamycin, cytoxan, oncovin, prednisone, and procarbazine), and COPP/ABV (cytoxan, oncovin, prednisone, procarbazine, adriamycin, bleomycin, and velban). More combinations are being explored in clinical trials. Reduction of radiation doses and fields is also being explored in pediatric trials. With very strict criteria, some patients are randomized to not receive radiation following chemotherapy.
NURSING ASSESSMENT
NURSING INTERVENTIONS
Staging Procedure
1. Provide preprocedural education to child and family (see Appendix F).
2. Prepare child for clinical staging procedures with age-appropriate approach (see Appendix F).
3. Assist and support child in collection of laboratory specimens.
4. Provide instruction, support, and family crisis intervention.
Radiation and/or Chemotherapy Phase
1. Provide sedation for radiation treatments if needed.
2. Monitor cardiorespiratory status during treatments.
3. Prepare for treatment-induced emergencies.
4. Assess for signs of extravasation of chemotherapeutic agents.
5. Monitor for signs and symptoms of infection.
7. Minimize side effects of radiotherapy and/or chemotherapy.
8. Provide ongoing emotional support to child and family (see Appendix F).
9. Refer to child life specialist to assist with continued coping strategies.
10. Provide ongoing education about treatment and follow-up care, medications—both chemotherapy and supportive care medications.
11. Refer family to social services for support and resource utilization.
12. If school age, refer to hospital or homebound teacher or obtain lessons for teaching.
Discharge Planning and Home Care
Instruct child and parents about home care management, including the following:
1. Signs of infection and guidelines on when to seek medical attention
2. Care of child’s central venous access device, including site care, dressing change, flushing, and emergency care
3. Medication administration (provide written information); review side effects and indications for medications
4. Adherence to treatment regimen and medical appointments, as well as future late effects follow-up
5. Proper nutrition for optimal weight gain and health maintenance
6. School attendance and/or activity restrictions
7. Potential behavioral changes in child and/or siblings
8. Stress importance of oral hygeine; discuss dental care and appointments while on therapy
9. Assess transportation needs
10. Provide and review staff phone numbers; that is, who to contact with questions, and who to contact for emergencies
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Max A, et al. Developing nursing care guidelines for children with Hodgkin’s disease. Eur J Oncol Nurs. 2003;7(4):253.
Yung L, et al. Long-term outcome in adolescents with Hodgkin’s lymphoma: Poor results using regimens designed for adults. Leuk Lymphoma. 2004;45(8):1579.
