ESSENTIALS OF DIAGNOSIS
Often painless lymphadenopathy.
Constitutional symptoms may or may not be present.
Pathologic diagnosis by lymph node biopsy.
Hodgkin lymphoma is characterized by lymph node biopsy showing Reed-Sternberg cells in an appropriate reactive cellular background (eFigure 13–34). The malignant cell is derived from B lymphocytes of germinal center origin.
Hodgkin lymphoma, mixed cellularity subtype, excisional lymph node biopsy. Shown here at high power to emphasize the larger Reed-Sternberg cells pathognomonic for Hodgkin disease as well as Reed-Sternberg variant cells (or lacunar cells). At low power (not shown) the mixed cellularity subtype of Hodgkin lymphoma shows a mixture of lymphocytes and histiocytes without significant fibrosis. (Reproduced, with permission, from Lichtman MA, Shafer MS, Felgar RE, Wang N. Lichtman's Atlas of Hematology. McGraw-Hill, 2016.)
There is a bimodal age distribution, with one peak in the 20s and a second over age 50 years. Most patients seek medical attention because of a painless mass, commonly in the neck. Others may seek medical attention because of constitutional symptoms such as fever, weight loss, or drenching night sweats, or because of generalized pruritus. An unusual symptom of Hodgkin lymphoma is pain in an involved lymph node following alcohol ingestion.
An important feature of Hodgkin lymphoma is its tendency to arise within single lymph node areas and spread in an orderly fashion to contiguous areas of lymph nodes. Late in the course of the disease, vascular invasion leads to widespread hematogenous dissemination.
Hodgkin lymphoma is divided into two subtypes: classic Hodgkin (nodular sclerosis, mixed cellularity, lymphocyte rich, and lymphocyte depleted) and non-classic Hodgkin (nodular lymphocyte predominant). Hodgkin lymphoma should be distinguished pathologically from other malignant lymphomas and may occasionally be confused with reactive lymph nodes seen in infectious mononucleosis, cat-scratch disease, or drug reactions (eg, phenytoin).
Patients undergo a staging evaluation to determine the extent of disease, including serum chemistries, whole-body PET/CT scan, and bone marrow biopsy. The staging nomenclature (Ann Arbor) is as follows: stage I, one lymph node region involved; stage II, involvement of two or more lymph node regions on one side of the diaphragm; stage III, lymph node regions involved on both sides of the diaphragm; and stage IV, disseminated disease with extranodal involvement. Disease staging is further categorized as "A" if patients lack constitutional symptoms or as "B" if patients have 10% weight loss over 6 months, fever, or drenching night sweats (or some combination thereof).
Chemotherapy is the mainstay of treatment for Hodgkin lymphoma, and ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine) remains the standard first-line regimen due to its manageable toxicity. The substitution of the antibody-drug conjugate brentuximab vedotin for bleomycin (AAVD) ...