Oncology · Lymphoma
The facts most likely to be tested
The hallmark histologic finding of Hodgkin Lymphoma is the Reed-Sternberg cell, which is a large, multinucleated cell with owl-eye inclusions.
Patients frequently present with a painless, rubbery lymphadenopathy in the cervical or supraclavicular region, often exacerbated by alcohol-induced pain.
Systemic B symptoms, including unexplained fever, night sweats, and weight loss, are critical prognostic indicators and are associated with a worse outcome.
The disease follows a bimodal age distribution, with peaks occurring in young adulthood (ages 15–35) and in older adults (over age 55).
Diagnosis is confirmed via excisional lymph node biopsy, as fine-needle aspiration is insufficient to evaluate the nodular architecture.
The Ann Arbor staging system is used to determine the extent of disease, which dictates the intensity of chemotherapy (ABVD regimen) and radiation therapy.
Long-term survivors of Hodgkin Lymphoma are at significantly increased risk for secondary malignancies, particularly breast cancer and lung cancer, due to prior radiation exposure.
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A 24-year-old male presents to the clinic with a two-month history of a painless, rubbery mass in his left neck. He reports a 10-pound unintentional weight loss and drenching night sweats over the same period. He notes that the mass becomes tender after he consumes a glass of wine at dinner. Physical examination reveals a 3 cm supraclavicular lymph node and no hepatosplenomegaly.
What is the most likely diagnosis?
Hodgkin Lymphoma
The patient's presentation of B symptoms, painless cervical lymphadenopathy, and the classic pathognomonic finding of alcohol-induced pain strongly points to Hodgkin Lymphoma.
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Etiology / Epidemiology
Bimodal age distribution (20s and >50s). Associated with Epstein-Barr virus infection.
Clinical Manifestations
Painless cervical/supraclavicular lymphadenopathy and Pel-Ebstein fever.
Diagnosis
Excisional lymph node biopsy showing Reed-Sternberg cells.
Treatment
ABVD regimen (Adriamycin, Bleomycin, Vinblastine, Dacarbazine).
Prognosis
Highly curable; 80-90% 5-year survival rate in early stages.
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Epidemiology & Etiology
Bimodal distribution peaks in young adults (20-30) and older adults (>50). Strong association with Epstein-Barr virus (EBV) in up to 50% of cases. Increased risk in patients with HIV or history of immunosuppression.
Pertinent Anatomy
Typically originates in a single lymph node chain, most commonly the cervical or supraclavicular nodes. Spreads in a predictable, contiguous fashion to adjacent lymph node groups.
Pathophysiology
Malignant transformation of B-lymphocytes leads to the formation of Reed-Sternberg cells. These cells secrete cytokines that recruit inflammatory cells, creating the characteristic inflammatory milieu. The disease is defined by the presence of these cells in a background of reactive lymphocytes.
Clinical Manifestations
Painless, rubbery lymphadenopathy is the hallmark. Patients may present with B symptoms: fever, night sweats, and weight loss >10%. Alcohol-induced pain at the site of lymphadenopathy is a rare but highly specific finding. Pel-Ebstein fever (cyclic fever) is a classic, though uncommon, presentation.
Diagnosis
Excisional lymph node biopsy is the gold standard for diagnosis. Histology must demonstrate Reed-Sternberg cells (large, multinucleated cells with 'owl-eye' appearance). PET/CT scan is required for staging to assess metabolic activity and disease extent.
Treatment
Standard of care is the ABVD regimen. Bleomycin carries a risk of pulmonary fibrosis, requiring baseline and serial pulmonary function tests. Radiation therapy is often used as an adjunct in localized disease. Secondary malignancies are a long-term concern for survivors.
Prognosis
Highly curable with modern chemotherapy and radiation. 80-90% 5-year survival rate. Long-term survivors require monitoring for cardiovascular disease and secondary cancers (breast, lung) due to prior radiation exposure.
Differential Diagnosis
Non-Hodgkin Lymphoma: usually presents with extranodal involvement and non-contiguous spread
Infectious Mononucleosis: acute onset with pharyngitis and positive heterophile antibody
Tuberculosis: associated with night sweats and pulmonary findings on CXR
Sarcoidosis: bilateral hilar adenopathy without Reed-Sternberg cells
Metastatic Carcinoma: usually older patients with a known primary site