Hodgkin’s disease is a type of lymphoma characterized by the presence of Reed-Sternberg cells surrounded by normal inflammatory cells on histologic examination. Disease presentation is usually in the form of painless lymphadenopathy, and there is a biphasic peak with disease presenting in both young adults and the elderly. Disease occurs more frequently in males than females, and Caucasians are more frequently afflicted than are African Americans. Persons with AIDs are at an increased risk for disease.
Aside from painless adenopathy, other presenting symptoms include: fever, night sweats, weight loss (greater than 10 percent of body weight), fatigue, pruritis, and pain localized to the affected lymph nodes after the ingestion of alcohol. When the fever is periodic with several days of normal temperatures in between bouts of fever, then the patient suffers from a phenomenon termed the Pel-Ebstein fever. The presence of systemic symptoms is important in the staging process. The presence of any one of these symptoms classifies a patient as B disease; whereas, disease without any of these symptoms classifies a person as A disease. Lymphomas which present with disease localized to the bone marrow may manifest symptoms consistent with anemia (shortness of breath, dyspnea on exertion, fatigue, etc) or thrombocytopenia (an increased tendency towards bleeding or ecchymosis). Disease which is localized to the spleen may present as left upper quadrant abdominal pain or fullness.
Diagnosis is established by the presence of Reed-Sternberg cells on histologic examination usually surrounded by T lymphocytes, eosinophils and other inflammatory cells. An excisional biopsy is required to ensure an adequate amount of tissue is available for examination. Inguinal lymph nodes should not be chosen as the site for biopsy if at all possible. The type of Hodgkin’s disease is determined by histologic exam. The different types include: lymphocyte-predominant, mixed cellularity, nodular sclerosis and lymphocyte-depleted. A more favorable prognosis is associated with lymphocyte-predominant disease, and conversely, lymphocyte-depleted disease carries a poor prognosis.
Laboratory abnormalities include an elevated LDH and hypergammaglobulinemia. The white blood cell differential may show monocytosis or eosinophilia. If lymphopenia is noted, this is considered a poor prognostic sign. Thrombocytopenia and anemia may also be noted on the CBC. The anemia may be secondary to chronic disease, autoimmune hemolytic anemia, or marrow invasion. Thrombocytopenia may be secondary to hypersplenism or marrow invaison. Hepatic involvement is indicated by abnormalities on liver function testing. A thorough laboratory evaluation should include a CBC, serum calcium level, renal function studies, and liver function studies to include an LDH determination.
Once the diagnosis is established by histology, CT scanning of the chest, abdomen and pelvis should be performed. If there are abnormalities noted on liver function testing or a suspicious lesion is noted in the liver, laparoscopic or CT guided liver biopsy should be performed. If available, a lymphangiogram should be performed when disease is associated with negative CT scanning. If CT scanning reveals a questionable lesion, gallium scanning may prove helpful. If the lesion is lymphoma, it will take up gallium. Gallium scanning is also useful when evaluating patients after treatment. Lesions may persist on CT scan after therapy, but these lesions may represent scar tissue or persistent disease. In this case, gallium scanning will differentiate between persistent disease (takes up gallium) and scar tissue (does not take up gallium). Also, marrow aspirate and biopsy of the bilateral iliac crests should be performed. After these studies have been performed the patient may be properly staged; however, some patients require laparotomy with biopsies and splenectomy for accurate staging.
Staging is as follows:
STAGE I: Characterized by only one set of affected lymph nodes either above or below the diaphragm. More than one node may be positive, but all nodes are from the same anatomic region.
STAGE II: Characterized by more than one set of lymph nodes are positive, but all positive nodes are on the same side of the diaphragm.
STAGE III: Characterized by two or more positive sets of lymph nodes with at least one positive set on each side of the diaphragm. The spleen counts as a lymph node, and persons with splenic involvement are identified as IIIS.
STAGE IV: Characterized by extranodal involvement (bone marrow, visceral organ or skin)
If the patient has no systemic symptoms of disease they are further characterized by the letter A following their stage of disease. If the patient manifests any systemic symptoms (fever, night sweats, weight loss) then they are further designated as B. Persons with splenic involvement are identified by an S after their staging level, and persons with localized extranodal involvement are designated by the letter E. For example, a person with one set of involved lymph nodes on each side of the diaphragm who also suffers from night sweats would be staged as IIIB.
Therapy is with either chemotherapy, radiation therapy or both. The two chemotherapy regimens available are MOPP (mechlorethamine, vincristine, procarbazine and prednisone) or ABVD (doxorubicin, bleomycin, vinblastine and dacarbazine). Complications of MOPP include sterility, bone marrow suppression, and the development of secondary leukemias. Doxorubicin induced cardiac toxicity and bleomycin induced pulmonary toxicity complicate treatment with ABVD. Consultation with a hematologist for treatment is necessary.