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
