Snapshot A 65-year-old man with mycosis fungoides presents with increased fatigue, low-grade fevers, and night sweats. Given his history, he is scared that "something is going on." His primary care physician does a thorough physical exam and finds several small, rubbery, mobile supraclavicular lymph nodes. They are nontender. A lymph node biopsy shows pathology of Reed-Sternberg cells. Introduction B-cell malignancy originating in lymphatic system Commonly early stage when diagnosed as opposed to non-Hodgkin lymphoma (NHL), where people are in later stage disease at diagnosis Types of Hodgkin lymphoma nodular sclerosing most common female predominance mixed cellularity lymphocyte-rich/predominant especially in < 35-year-olds lymphocyte-depleted especially in > 60-year-olds other systemic diseases Epidemiology Incidence bimodal distribution young adulthood > 55 years Demographics male > female except for nodular sclerosing type more common in Caucasians Risk factors infectious mononucleosis with EBV immunosuppression smoking mycosis fungoides ETIOLOGY Pathogenesis 50% of cases associated with EBV infection Reed-Sternberg cells CD15+ CD30+ Presentation Symptoms constitutional ("B") symptoms fever night sweats weight loss all caused by cytokines released from Reed-Sternberg cells contiguous spread persistent painless lymphadenopathy Physical exam nontender mass of localized, single group of nodes rubbery mobile cervical supraclavicular axillary potential SVC syndrome potential erythema nodosum studies Imaging for staging Lymph node biopsy Reed-Sternberg cells binucleate or bilobed, “owl-eyed” nuclei mixed cellularity type large inflammatory infiltrate with many eosinophils nodular sclerosing type diffuse band-like fibrosis with lacunar spaces Differential Diagnosis Non-Hodgkin lymphoma AIDS-related lymphadenopathy Infection Breast cancer Cat-scratch fever Treatment Based on staging Localized disease or early stage excisional biopsy chemotherapy radiation Late stage disease or anyone with B symptoms ABVD Adriamycin (doxorubicin) Bleomycin Vinblastine Dacarbazine Complications Tumor lysis syndrome hypocalcemia (most common) hyperkalemia hyperphosphatemia hyperuricemia SVC syndrome Minimal change disease Paraneoplastic syndromes calctriol secretion causes hypercalcemia From treatment risk of solid tumors (breast, thyroid, and lung) risk of premature coronary artery disease risk of infection doxorubicin – cardiomyopathy vincristine – neuropathy bleomycin – lung fibrosis Prognosis > 80% with treatment better than non-Hodgkin lymphoma lymphocyte-predominant = best prognosis lymphocyte-deplete = worst prognosis higher lymphocyte:RS cell ratio = better prognosis 5-30% chance of relapse