Chapter 105. Malignancies of Lymphoid Cells (Part 24) docx

Chapter 105. Malignancies of Lymphoid Cells (Part 24) A number of other late side effects from the treatment of Hodgkin's disease are well known. Patients who receive thoracic radiotherapy are at very high risk for the eventual development of hypothyroidism and should be observed for this complication; intermittent measurement of thyrotropin should be made to identify the condition before it becomes symptomatic. Lhermitte's syndrome occurs in ~15% of patients who receive thoracic radiotherapy. This syndrome is manifested by an "electric shock" sensation into the lower extremities on flexion of the neck. Infertility is a concern for all patients undergoing treatment for Hodgkin's disease. In both women and men, the risk of permanent infertility is age-related, with younger patients more likely to recover fertility. In addition, treatment with ABVD rather than MOPP increases the chances to retain fertility. Nodular Lymphocyte-Predominant Hodgkin's Disease Nodular lymphocyte-predominant Hodgkin's disease is now recognized as an entity distinct from classical Hodgkin's disease. Previous classification systems recognized that biopsies from a subset of patients diagnosed as having Hodgkin's disease contained a predominance of small lymphocytes and rare Reed-Sternberg cells. A subset of these patients have tumors with nodular growth pattern and a clinical course that varied from that of patients with classical Hodgkin's disease. This is an unusual clinical entity and represents <5% of cases of Hodgkin's disease. Nodular lymphocyte-predominant Hodgkin's disease has a number of characteristics that suggest its relationship to non-Hodgkin's lymphoma. These include a clonal proliferation of B cells and a distinctive immunophenotype; tumor cells express J chain and display CD45 and epithelial membrane antigen (ema) and do not express two markers normally found on Sternberg-Reed cells, CD30 and CD15. This lymphoma tends to have a chronic, relapsing course and sometimes transforms to diffuse large B cell lymphoma. The treatment of patients with nodular lymphocyte-predominant Hodgkin's disease is controversial. Some clinicians favor no treatment and merely close follow-up. In the United States, most physicians will treat localized disease with radiotherapy and disseminated disease with regimens utilized for patients with classical Hodgkin's disease. Regardless of the therapy utilized, most series report a long-term survival of >80%. Lymphoma-Like Disorders The most common condition that pathologists and clinicians might confuse with lymphoma is reactive, atypical lymphoid hyperplasia. Patients might have localized or disseminated lymphadenopathy and might have the systemic symptoms characteristic of lymphoma. Underlying causes include a drug reaction to phenytoin or carbamazepine. Immune disorders such as rheumatoid arthritis and lupus erythematosus, viral infections such as cytomegalovirus and EBV, and bacterial infections such as cat-scratch disease may cause adenopathy (Chap. 60). In the absence of a definitive diagnosis after initial biopsy, continued follow-up, further testing, and repeated biopsies, if necessary, are the appropriate approach rather than instituting therapy. Specific conditions that can be confused with lymphoma include Castleman's disease, which can present with localized or disseminated lymphadenopathy; some patients have systemic symptoms. The disseminated form is often accompanied by anemia and polyclonal hypergammaglobulinemia, and the condition has been associated with overproduction of interleukin 6, possibly produced by human herpesvirus 8. Patients with localized disease can be treated effectively with local therapy, while the initial treatment for patients with disseminated disease is usually with systemic glucocorticoids. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman's disease) usually presents with bulky lymphadenopathy in children or young adults. The disease is usually nonprogressive and self-limited, but patients can manifest autoimmune hemolytic anemia. Lymphomatoid papulosis is a cutaneous lymphoproliferative disorder that is often confused with anaplastic large cell lymphoma involving the skin. The cells of lymphomatoid papulosis are similar to those seen in lymphoma and stain for CD30, and T cell receptor gene rearrangements are sometimes seen. However, the condition is characterized by waxing and waning skin lesions that usually heal, leaving small scars. In the absence of effective communication between the clinician and the pathologist regarding the clinical course in the patient, this disease will be misdiagnosed. Since the clinical picture is usually benign, misdiagnosis is a serious mistake. Acknowledgment James Armitage was a coauthor of this chapter in prior editions, and substantial material from those editions has been included here Further Readings Ansel SM, Armitage JO: Management of Hodgkin lymphoma. Mayo Clin Proc 81:419, 2006 Armitage JO et al: Lymphoma 2006: Classification and treatment. Oncology 20:231, 2006 [PMID: 16629256] Brepoels L et al: PET and PET/CT fo r response evaluation in lymphoma: Current practice and developments. Leuk Lymphoma 48:270, 2007 [PMID: 17325886] Harris NL et al: World Health Organization classification of neoplastic diseases of the hematopoietic and lymphoid tissues: Report of the Cl inical Advisory Committee Meeting, Airlie House, Virginia, November, 1997. J Clin Oncol 17:3835, 1999 [PMID: 10577857] Sehn LH et al: The revised International Prognostic Index (R- IPI) is a better predictor of outcome than the standard IPI for patients with diffuse large B- cell lymphoma treated with R-CHOP. Blood 109:1857, 2007 [PMID: 17105812] Urba WJ, Longo DL: Hodgkin's disease. N Engl J Med 326:678, 1992 [PMID: 1736106] . Chapter 105. Malignancies of Lymphoid Cells (Part 24) A number of other late side effects from the treatment of Hodgkin's disease are well known that biopsies from a subset of patients diagnosed as having Hodgkin's disease contained a predominance of small lymphocytes and rare Reed-Sternberg cells. A subset of these patients have tumors. a number of characteristics that suggest its relationship to non-Hodgkin's lymphoma. These include a clonal proliferation of B cells and a distinctive immunophenotype; tumor cells express