Hodgkin’s Disease

The Lymphomas

The lymphomas are neoplasms of cells of lymphoid origin. These tumors usually start in lymph nodes but can involve lymphoid tissue in the spleen, the gastrointestinal tract (eg, the wall of the stomach), the liver, or the bone marrow. They are often classified according to the degree of cell differentiation and the origin of the predominant malignant cell. Lymphomas can be broadly clas-sified into two categories: Hodgkin’s disease and non-Hodgkin’s lymphoma (NHL).

HODGKIN’S DISEASE

Hodgkin’s disease is a relatively rare malignancy that has an im-pressive cure rate. It is somewhat more common in men than women and has two peaks of incidence: one in the early 20s and the other after 50 years of age. Unlike other lymphomas, Hodgkin’s disease is unicentric in origin in that it initiates in a sin-gle node. The disease spreads by contiguous extension along the lymphatic system. The cause of Hodgkin’s disease is unknown, but a viral etiology is suspected. In fact, fragments of the Epstein-Barr virus have been found in 40% to 50% of patients; this occurs more commonly in the younger patient population (Weiss, 2000). There is a familial pattern associated with Hodgkin’s disease: first-degree relatives have a higher-than-normal frequency of the dis-ease. There is no increased incidence documented for non-blood relatives (eg, spouses).

The malignant cell of Hodgkin’s disease is the Reed-Sternberg cell, a gigantic tumor cell that is morphologically unique and is thought to be of immature lymphoid origin. It is the pathologic hallmark and essential diagnostic criterion for Hodgkin’s disease. However, the tumor is very heterogeneous and may actually con-tain few Reed-Sternberg cells. Repeated biopsies may be required to establish the diagnosis.

Hodgkin’s disease is customarily classified into five subgroups based on pathologic analyses that reflect the natural history of the malignancy and suggest the prognosis. For example, when lym-phocytes predominate, with few Reed-Sternberg cells and minimal involvement of the lymph nodes, the prognosis is much more fa-vorable than when the lymphocyte count is low and the lymph nodes are virtually replaced by tumor cells of the most primitive type. The majority of patients with Hodgkin’s disease have the types currently designated “nodular sclerosis” or “mixed cellularity.” The nodular sclerosis type tends to occur more often in young women, at an earlier stage but with a worse prognosis than the mixed cellu-larity subgroup, which occurs more commonly in men and causes more constitutional symptoms but has a better prognosis.

Clinical Manifestations

Hodgkin’s disease usually begins as a painless enlargement of one or more lymph nodes on one side of the neck. The individual nodes are painless and firm but not hard. The most common sites for lymphadenopathy are the cervical, supraclavicular, and medi-astinal nodes; involvement of the iliac or inguinal nodes or spleen is much less common. A mediastinal mass may be seen on chest x-ray; occasionally, the mass is large enough to compress the trachea and cause dyspnea. Pruritus is common; it can be extremely dis-tressing, and the cause is unknown. Approximately 20% of patients experience brief but severe pain after drinking alcohol (Cavalli, 1998). The pain is usually at the site of the Hodgkin’s disease; again, the cause is unknown.

All organs are vulnerable to invasion by Hodgkin’s disease. The symptoms result from compression of organs by the tumor, such as cough and pulmonary effusion (from pulmonary infiltrates), jaundice (from hepatic involvement or bile duct obstruction), ab-dominal pain (from splenomegaly or retroperitoneal adenopathy), or bone pain (from skeletal involvement). Herpes zoster infections are common. A cluster of constitutional symptoms has important prognostic implications. Referred to as “B symptoms,” they include fever (without chills), drenching sweats (particularly at night), and unintentional weight loss of more than 10%. “B symptoms” are found in 40% of patients and are more common in advanced disease.

A mild anemia is the most common hematologic finding. The WBC count may be elevated or decreased. The platelet count is typically normal, unless the tumor has invaded the bone marrow, suppressing hematopoiesis. The erythrocyte sedimentation rate(ESR) and the serum copper level are used by some clinicians toassess disease activity. Patients with Hodgkin’s disease have im-paired cellular immunity, as evidenced by an absent or decreased reaction to skin sensitivity tests (eg, Candida, mumps).

Assessment and Diagnostic Findings

Because many manifestations are similar to those occurring with infection, diagnostic studies are performed to rule out an infectious origin for the disease. The diagnosis is made by means of an exci-sional lymph node biopsy and the finding of the Reed-Sternberg cell. Once the diagnosis is confirmed and the histologic type is es-tablished, it is necessary to assess the extent of the disease, a process referred to as staging.During the health history, the nurse should assess for any “B symptoms.” Physical examination requires a careful, systematic evaluation of the lymph node chains, as well as the size of the spleen and liver. A chest x-ray and a CT scan of the chest, abdomen, and pelvis are crucial to identify the extent of lymphadenopathy within these regions. Laboratory tests include CBC, platelet count, ESR, and liver and renal function studies. A bone marrow biopsy is per-formed if there are signs of marrow involvement, and some physi-cians routinely perform bilateral biopsies. Bone scans may be performed to identify any involvement in these areas. A staging la-parotomy and lymphangiography are no longer considered manda-tory, primarily because of the accuracy of CT.

Medical Management

The general intent in treating Hodgkin’s disease, regardless of stage, is cure. Treatment is determined primarily by the stage of the disease, not the histologic type; however, extensive research is ongoing to target treatment regimens to histologic subtypes or prognostic features. Traditionally, early Hodgkin’s disease was treated by a staging laparotomy followed by radiation therapy. Re-cent data show improved results and decreased complications with a short course (2 to 4 months) of chemotherapy followed by radi-ation therapy in certain subsets of early-stage disease (IA and IIA); patients with early-stage disease and good prognostic features may receive radiation therapy alone (Hoppe et al., 2000). Combina-tion chemotherapy, for example with doxorubicin (Adriamycin), bleomycin (Blenoxane), vinblastine (Velban), and dacarbazine (DTIC), referred to as ABVD, is now the standard treatment for more advanced disease (stages III and IV and all B stages).

Radiation therapy is still very useful for patients with exten-sive adenopathy (often termed bulky disease). In this group, residual disease often persists after the chemotherapy treatment is finished; radiation therapy to the areas of remaining adenopathy has been shown to improve survival.

Even when Hodgkin’s disease does recur, the use of high doses of chemotherapeutic agents, followed by autologous BMT or stem cell transplantation (PBSCT), can be very effective in controlling the disease and extending survival time.

Long-Term Complications of Therapy

Much is now known about the long-term effects of chemotherapy and radiation therapy, primarily from the large numbers of peo-ple who were cured of Hodgkin’s disease by these treatments. The various complications of treatment are listed in Chart 33-12. Risk factors for other cancers should be assessed, and long-term sur-veillance is crucial. The potential development of a second malig-nancy is obviously of concern to patients, and this potential should be addressed with the patient when treatment decisions are made. However, it is important to consider that Hodgkin’s disease is cur-able. Revised treatment approaches are aimed at diminishing the risk for complications without sacrificing the potential for cure.