Lymphoma: Diagnosis and Treatment
Am Fam Physician. 2020 Jan 1;101(1):34-41.
Patient information: See related handout on lymphoma, written by the authors of this article.
Author disclosure: No relevant financial affiliations.
Lymphoma is a group of malignant neoplasms of lymphocytes with more than 90 subtypes. It is traditionally classified broadly as non-Hodgkin or Hodgkin lymphoma. Approximately 82,000 new U.S. patients are diagnosed with lymphoma annually. Any tobacco use and obesity are major modifiable risk factors, with genetic, infectious, and inflammatory etiologies also contributing. Lymphoma typically presents as painless adenopathy, with systemic symptoms of fever, unexplained weight loss, and night sweats occurring in more advanced stages of the disease. An open lymph node biopsy is preferred for diagnosis. The Lugano classification system incorporates symptoms and the extent of the disease as shown on positron emission tomography/computed tomography to stage lymphoma, which is then used to determine treatment. Chemotherapy treatment plans differ between the main subtypes of lymphoma. Non-Hodgkin lymphoma is treated with CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) with or without rituximab (R-CHOP), bendamustine, and lenalidomide. Hodgkin lymphoma is treated with combined chemotherapy with ABVD (doxorubicin, bleomycin, vinblastine, and dacarbazine), Stanford V (a chemotherapy regimen consisting of mechlorethamine, doxorubicin, vinblastine, vincristine, bleomycin, etoposide, and prednisone), or BEACOPP (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, and prednisone) with radiotherapy. Subsequent chemotherapy toxicities include neuropathy, cardiotoxicity, and secondary cancers such as lung and breast, and should be considered in the shared decision-making process to select a treatment regimen. Once remission is achieved, patients need routine surveillance to monitor for complications and relapse, in addition to age-appropriate screenings recommended by the U.S. Preventive Services Task Force. Patients should receive a 13-valent pneumococcal conjugate vaccine followed by a 23-valent pneumococcal polysaccharide vaccine at least eight weeks later with additional age-appropriate vaccinations because lymphoma is an immunosuppressive condition. Household contacts should also be current with their immunizations.
Lymphoma represents a heterogeneous group of malignant neoplasms of lymphocytes, which can involve lymphatic tissue, bone marrow, or extranodal sites. The World Health Organization’s classification system identifies more than 90 different subtypes (Table 1).1,2 The initial stratification is derived from B-cell, T-cell, or natural killer cell origin. Further classification of distinct lymphoma subtypes is beyond the scope of this article; however, they are ultimately each defined by morphology, immunopheno-type, genetic, molecular, and clinical features.1,3 This article will focus on the types of lymphoma traditionally classified as non-Hodgkin or Hodgkin.
SORT: KEY RECOMMENDATIONS FOR PRACTICE
| Clinical recommendation | Evidence rating | Comments |
|---|---|---|
Open lymph node biopsy should be used to definitively diagnose lymphoma.14,15 | C | Expert opinion and clinical review articles |
Positron emission tomography/computed tomography should be used to determine the staging of the lymphoma.19 | C | Expert opinion and clinical review article |
Patients with lymphoma should have intensive follow-up surveillance for the first two years following remission.40 | C | Expert opinion and clinical review article |
A 13-valent pneumococcal conjugate vaccine (Prevnar 13), followed by a 23-valent pneumococcal polysaccharide vaccine (Pneumovax 23) at least eight weeks later and then again at least five years later, should be administered following lymphoma treatment.44,45 | C | Expert opinion and guidelines |
A = consistent, good-quality patient-oriented evidence; B = inconsistent or limited-quality patient-oriented evidence; C = consensus, disease-oriented evidence, usual practice, expert opinion, or case series. For information about the SORT evidence rating system, go to https://www.aafp.org/afpsort.
SORT: KEY RECOMMENDATIONS FOR PRACTICE
| Clinical recommendation | Evidence rating | Comments |
|---|---|---|
Open lymph node biopsy should be used to definitively diagnose lymphoma.14,15 | C | Expert opinion and clinical review articles |
Positron emission tomography/computed tomography should be used to determine the staging of the lymphoma.19 | C | Expert opinion and clinical review article |
Patients with lymphoma should have intensive follow-up surveillance for the first two years following remission.40 | C | Expert opinion and clinical review article |
A 13-valent pneumococcal conjugate vaccine (Prevnar 13), followed by a 23-valent pneumococcal polysaccharide vaccine (Pneumovax 23) at least eight weeks later and then again at least five years later, should be administered following lymphoma treatment.44,45 | C | Expert opinion and guidelines |
A = consistent, good-quality patient-oriented evidence; B = inconsistent or limited-quality patient-oriented evidence; C = consensus, disease-oriented
References
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