Definition
A.Malignancies that develop from lymph nodes and lymphoid tissues are broadly classified into Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL).
B.HL, which originates in germinal center or postgerminal center B-lymphocytes, is characterized by the presence of a distinctive type of giant cell called a Reed–Sternberg cell in a background of reactive cells.
Incidence
A.HL accounts for approximately 10% of all lymphomas and approximately 0.6% of all cancers diagnosed annually in the developed world.
B.Median age at diagnosis is 39 years.
C.In 2016, an estimated 8,500 patients were diagnosed with HL, and an estimated 1,120 patients died of HL.
D.In the United States, the most common subtype of HL is classical HL, followed by mixed cellularity, lymphocyte-rich, and lymphocyte-depleted.
Pathogenesis
A.HL occurs due to the clonal proliferation of malignant Hodgkin/Reed–Sternberg cells in a background of reactive cells.
B.The proliferation of these malignant cells causes lymphadenopathy and enlargement of lymphoid tissue/organs (e.g., spleen).
C.Lymphoma generally spreads to contiguous lymph nodes following lymph vessels.
Predisposing Factors
A.Epstein–Barr virus (EBV) infection.
B.Immunosuppression (e.g., patients with HIV infection or long-term immunosuppressant use).
C.Family history of HL.
D.Most patients who develop HL have no identifiable risk factors.
Subjective Data
A.Common complaints/symptoms.
1.Painless lymphadenopathy, most commonly in the neck, axilla, or groin, is the most common presenting complaint. Occasionally, lymph nodes can become painful after consuming alcohol.
2.B-symptoms: Unintentional weight loss, fever, and night sweats.
B.Common/typical scenario.
1.Other signs and symptoms.
a.Generalized pruritus.
b.Fatigue.
c.Lack of appetite.
d.Cough, difficulty breathing, or chest pain secondary to large mediastinal mass or lymphadenopathy. Often a mediastinal mass will be discovered incidentally on a routine chest radiograph.
C.Family and social history.
1.Elicit presence or absence and duration of symptoms.
2.Ask about previous malignancy, prior treatment with chemotherapy or radiation therapy, history of immunosuppressive illnesses such as HIV, and family history of malignancy.
D.Review of systems: Determine the patient’s performance status, as this can impact future treatment options.
Physical Examination
A.A complete physical examination, including vital signs, should be performed.
B.Special attention should be paid to the size and number of palpable peripheral lymph nodes and presence or absence of hepatosplenomegaly.
C.Comprehensive neurological examination should be performed to assess for central nervous system (CNS) involvement.
Diagnostic Tests
A.Definitive diagnosis is made by lymph node biopsy. An excisional biopsy is preferred for diagnosis but often core biopsy of an involved node is sufficient.
B.Complete workup should include complete blood count (CBC), erythrocyte sedimentation rate (ESR), comprehensive metabolic panel (CMP), lactate dehydrogenase (LDH), pregnancy test in women with childbearing potential, and HIV serology.
C.Clinical staging, including the following evaluations, should also be completed.
1.Full-body PET/CT scan.
2.Bilateral bone marrow biopsy/aspiration should be considered if the patient has pancytopenia.
3.Lumbar puncture and/or dedicated brain imaging, only if CNS involvement is suspected.
D.HL is staged per the Ann Arbor Staging.
E.Other important tests to consider include pulmonary function tests and echocardiogram, as they will likely be needed for assessment prior to chemotherapy or radiation therapy.
Differential Diagnosis
A.HIV.
B.Other solid tumors.
C.NHL
D.Any disease with lymphadenopathy needs to be considered.
Evaluation and Management Plan
A.General plan.
1.Treatment based on the stage of the disease at diagnosis, but can involve chemotherapy, radiation therapy, and immunotherapy, either alone or in combination.
2.Several chemotherapy regimens exist for the initial treatment of HL.
3.High-dose chemotherapy followed by autologous or allogeneic stem cell transplant may be indicated for refractory or recurrent disease.
Follow-Up
A.Based on individual needs and individual oncologists.
B.Basic schedule includes:
1.Visits every 3 to 6 months for first 2 years, every 6 to 12 months in years 3 to 5, and annually after year 5.
2.Usual tests are physical examination, blood work, imaging as indicated, and surveillance of symptom changes.
Consultation/Referral
A.Immediate referral to an oncologist should be made when HL is suspected or diagnosed.
Special/Geriatric Considerations
A.HL is one of the most curable malignancies in adults; however, survival rates in elderly patients are significantly lower than in younger patients.
Bibliography
Cheson, B. D., Fisher, R. I., Barrington, S. F., Cavalli, F., Schwartz, L. H., Zucca, E., & Lister, T. A. (2014). Recommendations for initial evaluation, staging, and response assessment of Hodgkin and non-Hodgkin lymphoma: The Lugano classification. Journal of Clinical Oncology, 32(27), 3059–3068.
National Cancer Institute. (2016, September 12). Surveillance, epidemiology, and end results program. Retrieved from https://seer.cancer.gov/faststats/selections.php?series=cancer
Siegel, R., Ma, J., Zou, Z., & Jemal, A. (2014). Cancer statistics, 2014. CA: A Cancer Journal for Clinicians, 64(1), 9–29.