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365 Article 1 Clock Hour Hodgkin’s Disease Gerard P. Boe Introduction Hodgkin’s disease is a malignancy that originates in the lymphatic system. The disorder usually begins in the lymph nodes or the spleen but can also be present in other areas. To understand the changes that occur in lymphoma, it is necessary to provide a short look at the lymphatic system. Lymph is a substance similar to plasma which circulates through a system of vessels similar to the blood circulation system. During normal metabolism, a small amount of the fluid portion of the blood filters out of the capillaries into the surrounding tissues. The lymphatic system helps maintain a balanced internal tissue environment by removing excess fluid as lymph. The fluid is drained through afferent vessels into small bean-shaped lymph nodes. Gerard P. Boe, PhD., CLC(AMT), MT(AMT), Executive Director of AMTIE, Editor of Journal of Continuing Education Topics & Issues, and Chair, AMT CLC Evaluation Committee Function of the Lymphatic System The primary function of the lymphatic system is to provide an immune defense. This complex system can differentiate between self and foreign molecules (antigens) and is able to recognize antigens it previously encountered and mount a defense. In addition to these outside antigens, the lymphatic system is capable of removing aged and damaged (senescent) cells. During fetal development, lymphatic precursors originate first in the fetal liver and later in the bone marrow. At this time in their development, some of the cells migrate to the thymus and become T-cells while those that remain in the liver or bone marrow become either B-cells or natural killer cells (NK). The thymus and bone marrow are considered to be primary lymphoid organs because they are maturation sites for the cells, which move into the secondary lymphoid organs where they serve to protect the body against foreign antigens. An antigen is any substance which the body may consider to be foreign. They (antigens) may be viruses, tumor cells, bacteria or fungi. The interaction of the T-cells and B-cells protect the body against these invaders and is called the immune response. Lymphatic Alterations in Hodgkin’s Disease Changes that occur in this disease can be categorized into three areas — anatomic, histologic and immune function. Anatomic Changes: The physical changes that may occur are enlarged spleen and lymph nodes. When the spleen is involved, it most often indicates spread through the hematologic system and may also include the liver and bone marrow. Histological Changes: There may be a significant presence of large Reed-Sternberg or Hodgkin’s cells. There may be a depletion of lymphocytes and an increase in eosinophils. The Reed-Sternberg cells are binucleated or multinucleated and must be present in order for the diagnosis of Hodgkin’s disease to be made. Immune Function: Cellular immunity is defective in Hodgkin’s lymphoma. Even for several years after successful treatment, the production and function of the T-cells may be impaired. The abnormalities include a decreased natural killer cell toxicity, suppressor monocyte activity and possibly delayed cutaneous hypersensitivity. The function of B-cells is usually normal in untreated patients with Hodgkin’s disease. Signs and Symptoms The severity of the disease is often proportional to the number of systems or organs involved. It may be localized or it may present itself in a number of different systems. Symptoms may appear in any one or more of the following systems: Lymphatic System — Many patients have enlarged lymph nodes. Frequently, the nodes are located in the cervical and the supraclavicular area but they may also be seen in the axillary, mediastinal and inguinal reagions. Pain in the lymph nodes after the ingestion of alcohol is found in about 10% of patients and can be said to be specific for Hodgkin’s. Renal system — Troubles in the renal system are often a complication of advanced disease. Metabolic continued pn page 77 (after insert ) 76 April 2010 • Continuing Education Topics & Issues abnormalities, such as abnormal uric acid levels, may occur. Bone Marrow — Bone marrow involvement occurs in only about 10% of untreated cases. Bone marrow involvement may demonstrate the following characterstics: increase in granulocytes, monocytes, and eosinophils and there may be areas of fibrosis along with the abnormal infiltration of lymphocytes and macrophages without the presence of HodgkinReed cells. Gastrointestinal — Patients may complain of abdominal pain, vomting and nausea. If the spleen or abdominal lymph nodes are involved, there may be other vague symptoms and GI bleeding. Diagnosis and Treatment Hodgkin's may occur from early childhood to old age. Increased numbers of cases of some types may be noted in patients between 15 and 35 and then again after 50 years of age. Males seem to be more susceptible than females. When Hodgkin's disease is suspected, microscopic histological examination of the lymph nodes through a biopsy helps to classify the Hodgkin's. The histology of Hodgkin's can vary from patient to patient; it is important to determine the classification and the treatment as well as the prognosis. Treatment modalities for individuals with Hodgkin's disease are based on age, classification and the overall condition of the patient. One or more of the following methods may be used: • Chemotherapy • Irradiation • Chemotherapy and irradiation • Bone marrow transplant Summary Lymphomas are malignancies that originate in the lymphatic system. Usually originating in the lymph nodes, these disorders can infiltrate in areas such as lungs, bone, testes and brain. Hodgkin's disease is one of the two major categories of lymphomas. The thymus and bone marrow are categorized as primary lymphoid organs because they function as differentiation and maturation sites. The lymphoid organs serve as sites where lymphocytes protect the body against antigens. The interaction of T and B cells to protect the body from the "foreign invaders" (antigens) is called the immune response. The cause or causes of Hodgkin’s have not been identified. Some researchers have voiced the opinion that it is heterogeneous and possibly represents more than one disease. References Devita, Jr., Vincent T., et. al., Cancer:Principles and Practice of Oncology, 5th ed, 2001 Beutler, Ernest, et al, Williams Hematology 6th ed, 2001 Levine, Sandra M., and Mochamuk, Robert S., MD, Hodgkin's Disease: Reducing Treatment Toxicity and Secondary Malignancies, Annual Meeting of American Society of Clinical Oncology, May 2001. Ballanti, Joseph A. ed., Clinical Immunology, W.B Saunders Co., Philadelphia, 1994 Continuing Education Topics & Issues • April 2010 77 365 Article 1 Clock Hour Questions for STEP Participants You can submit your answers either online or manually. To submit your answers online, go to www.amt1.com, click on AMT Store on the top navigation bar. Click on STEP Online, then select the article number and purchase the relevant test (member fee is $3/online test, no cost for download of article — don't forget to login with your member ID to receive the discounted $3 online test fee; nonmember fee is $5 for download of article, $10 for online test). If you wish to submit answers manually (only available to AMT members — member fee is $6/manual test), please submit answers on official AMT answer sheets only. Request answer sheets by email: [email protected]; by fax: 847-823-0458; or by mail: STEP Program Answer Sheets, American Medical Technologists, 10700 W. Higgins Road, Suite 150, Rosemont, IL 60018. When submitting answers manually, please be sure to include all the required information on the answer sheet and the $6 processing fee per article. In the following, choose the one best answer for each question. 1 Lymph is similar in composition to: A. blood B. plasma C. spinal fluid D. pleural fluid 2 Which of the following are primary lymphoid organs? 1. bone marrow 2. lymph nodes 3. thymus 4. tonsils A. 1, 2, 3 B. 1 and 3 C. 2 and 4 D. 4 only 3 In Hodgkin’s, metabolic disorders may occur and produce abnormal levels of uric acid. A. True B. False 4 Females seem to be more susceptible to Hodgkin’s disease than males. A. True B. False 5 In addition to providing antimmune response, the lymphatic system removes senescent cells. A. True B. False 78 April 2010 • Continuing Education Topics & Issues 6 At the time of their maturation, some cells move to the thymus and become T-cells. A. True B. False 7 In order to make the diagnosis of Hodgkin’s, multinucleated Reed-Sternberg cells must be present. A. True B. False 8 The lymphatic system helps maintain a balanced internal tissue environment by removing excess fluid. A. True B. False 9 Anatomic changes in Hodgkin’s may be in the form of an enlarged spleen and lymph nodes. A. True B. False 10 Cellular immunity is defective in Hodgkin’s. A. True B. False