Lymphoma for the Internist
Presenter: Dr. Sutamtewagul
The cause of some lymphoma subtypes is underlying infection, including Helicobacter pylori, Epstein-Barr virus, HIV, human T-cell lymphotrophic virus type-1, and hepatitis B and C virus infections.
In addition to chronic inflammation caused by infectious agents, genetic factors and occupational risk factors predisposing to lymphoma include exposure to herbicides, chlorinated organic compounds, and other fertilizing material used in farming.
Patients with small, soft, freely moveable lymph nodes that are limited to one or two adjacent sites and who have no other significant history or physical examination findings can be followed with serial examinations and require no laboratory studies or imaging.
Persistent or enlarging lymphadenopathy, particularly when associated with systemic symptoms, may require further assessment, including a chest radiograph, a complete blood count with differential, a serum chemistry panel, and assessment for viral infections.
Excisional biopsy and core biopsy for deep lymphadenopathy are appropriate for diagnosing lymphoma; however, fine-needle aspiration should not be used.
The Ann Arbor staging criteria can be used for determining extent of disease for most forms of lymphoma.
Use of International Prognostic Index (IPI) scores can help determine the prognosis of patients with diffuse large B-cell lymphoma (revised IPI), mantle cell lymphoma (MIPI), and follicular lymphoma (FLIPI), although new therapies, in addition to treatment with rituximab, have lessened the predictive value of these indices.
The clinical course of patients with follicular lymphoma is usually indolent, with most patients having lymphadenopathy but no symptoms at presentation, and some patients requiring no therapy for decades.
Allogeneic hematopoietic stem cell transplantation remains the only curative therapy for follicular lymphoma but is associated with a significant risk for morbidity and mortality.
In patients with mucosa-associated lymphoid tissue lymphoma, complete remission is achieved in most patients with limited disease without chemotherapy after completion of antimicrobial therapy directed against Helicobacter pylori infection and concomitant proton pump inhibitor therapy.
For mucosa-associated lymphoid tissue lymphoma that is not localized to the stomach or is not responsive to Helicobacter pylori infection eradication, treatment includes surgical removal, involved-field radiation therapy, and, when indicated, anti-CD20–directed therapy with rituximab combined with multiagent chemotherapy.
Most patients with diffuse large B-cell lymphoma present with advanced (stage III and IV) disease, have B symptoms (fever, night sweats, or weight loss), and will experience rapid disease progression without therapy.
Standard therapy for all patients with diffuse large B-cell lymphoma, regardless of stage or prognosis, is rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP).
Most patients with mantle cell lymphoma present with advanced disease characterized by lymphadenopathy, weight loss, and occasionally fever, and have diffuse sites of involvement, including the gastrointestinal tract, bone marrow, and bloodstream.
Allogeneic hematopoietic stem cell transplantation, although associated with a significant risk of morbidity and mortality, can produce long-term remissions in patients with mantle cell lymphoma, even in those with relapsed disease.
Hodgkin lymphoma is curable in most patients, even those with advanced disease.
Patients with stage I and II Hodgkin lymphoma without B symptoms can be treated effectively with radiation alone or radiation combined with a short course of chemotherapy, whereas those with stage III and IV disease or with B symptoms, regardless of stage, usually require a full course of chemotherapy.
Patients with recurrent chemotherapy-sensitive Hodgkin lymphoma usually are candidates for autologous hematopoietic stem cell transplantation, whereas those with disease resistant to salvage chemotherapy may achieve long-term remissions with allogeneic hematopoietic stem cell transplantation.
Early-stage cutaneous T-cell non-Hodgkin lymphoma is treated with topical glucocorticoids, retinoids (when needed), and psoralen and ultraviolet light with interferon alfa.
Advanced-stage cutaneous T-cell non-Hodgkin lymphoma is treated with chemotherapy, purine analogs, histone deacetylase inhibitors, and monoclonal antibodies.
Allogeneic hematopoietic stem cell transplantation may be curative in young patients with an appropriate donor.