From the Guidelines
Splenomegaly in lymphoma should be managed by treating the underlying lymphoma with appropriate chemotherapy regimens, with splenectomy reserved for rare cases with massive symptomatic splenomegaly or refractory disease. The presence of splenomegaly in lymphoma is a common finding, particularly in non-Hodgkin lymphomas, and can be associated with various subtypes, including mantle cell lymphoma, as noted in a study published in the Journal of Hematology and Oncology 1. This study highlights the heterogeneous clinical course of mantle cell lymphoma, which can include splenomegaly as a presenting feature.
Management of Splenomegaly
The primary approach to managing splenomegaly in lymphoma involves treating the underlying disease. Chemotherapy regimens such as R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone) for diffuse large B-cell lymphoma or bendamustine plus rituximab for follicular lymphoma are commonly used. As the lymphoma responds to treatment, the spleen typically decreases in size.
Role of Splenectomy
Splenectomy is considered in rare cases, such as those with massive symptomatic splenomegaly (>10 cm below the costal margin) and accompanied by low-level bone marrow infiltration, as indicated by guidelines for hairy cell leukemia 1. It can also be an option for patients with progressive disease during pregnancy or those refractory to nucleoside analogues and IFN-α. However, systemic therapy should not be initiated sooner than 6 months after splenectomy to allow for full benefits of the treatment.
Monitoring and Complications
Patients with lymphoma-associated splenomegaly should be monitored for potential complications, including:
- Cytopenias due to hypersplenism
- Splenic infarction
- Rarely, splenic rupture, which presents as acute abdominal pain and requires emergency intervention. Vaccination against Haemophilus influenza and pneumococcus before splenectomy is recommended 1.
Overall, the management of splenomegaly in lymphoma focuses on treating the underlying disease, with splenectomy and other interventions reserved for specific, severe cases.
From the Research
Splenomegaly with Lymphoma
- Splenomegaly is a common symptom in patients with lymphoma, particularly in those with splenic marginal zone lymphoma (SMZL) 2 or primary splenic diffuse large B-cell lymphoma (PS-DLBCL) 3.
- The treatment of splenomegaly with lymphoma depends on the type of lymphoma and the patient's overall health.
- For patients with SMZL, rituximab monotherapy or in combination with chemotherapy has shown remarkable responses, with overall response rates of 88-100% 2, 4.
- In patients with PS-DLBCL, splenectomy at diagnosis has been shown to improve survival, particularly in those with early-stage disease 3.
- The R-CHOP regimen, which includes rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone, is a standard treatment for diffuse large B-cell lymphoma (DLBCL) and has been shown to cure approximately 70% of patients 5, 6.
Treatment Options
- Rituximab monotherapy or in combination with chemotherapy is a treatment option for patients with SMZL 2, 4.
- Splenectomy at diagnosis may be beneficial for patients with PS-DLBCL, particularly those with early-stage disease 3.
- The R-CHOP regimen is a standard treatment for DLBCL and has been shown to be effective in curing approximately 70% of patients 5, 6.
- Dose reduction or dose intensification of the R-CHOP regimen may be considered in certain patients, such as the elderly or those with comorbidities 6.
Prognosis
- The prognosis for patients with splenomegaly and lymphoma depends on the type of lymphoma and the patient's overall health.
- Patients with SMZL who receive rituximab monotherapy or in combination with chemotherapy have a favorable prognosis, with overall response rates of 88-100% 2, 4.
- Patients with PS-DLBCL who undergo splenectomy at diagnosis have improved survival rates, particularly those with early-stage disease 3.
- Patients with DLBCL who receive the R-CHOP regimen have a cure rate of approximately 70% 5, 6.