From the Guidelines
Splenic lymphoma typically presents with symptoms such as abdominal discomfort or fullness in the left upper quadrant, fatigue, unintentional weight loss, night sweats, and fever, and treatment options depend on the specific type of lymphoma, stage, and patient factors, with a watch-and-wait approach, splenectomy, or systemic therapy involving rituximab and chemotherapy regimens being considered. The presentation of spleen lymphoma can vary, but common symptoms include abdominal discomfort, fatigue, weight loss, night sweats, and fever, as noted in various studies 1.
- Key symptoms:
- Abdominal discomfort or fullness in the left upper quadrant
- Fatigue
- Unintentional weight loss
- Night sweats
- Fever
- Physical examination findings:
- Splenomegaly (enlarged spleen)
- Anemia, thrombocytopenia, or leukopenia due to hypersplenism Treatment options for splenic lymphoma depend on the specific type of lymphoma, stage, and patient factors, with a focus on minimizing morbidity, mortality, and improving quality of life 1.
- For indolent splenic lymphomas like splenic marginal zone lymphoma:
- Watch-and-wait approach for asymptomatic patients
- Splenectomy for diagnostic confirmation and symptom relief, particularly for cytopenias
- Systemic therapy involving rituximab (375 mg/m² weekly for 4-8 weeks), either alone or combined with chemotherapy regimens such as bendamustine, CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone), or purine analogs like fludarabine
- For aggressive lymphomas like diffuse large B-cell lymphoma involving the spleen:
- R-CHOP (rituximab plus CHOP) is the standard treatment, typically administered every 21 days for 6 cycles
- Radiation therapy may be used in select cases, particularly for localized disease The treatment approach should be individualized based on the patient's age, comorbidities, lymphoma subtype, and disease extent, with management ideally coordinated by a multidisciplinary team including hematologists, oncologists, and surgeons 1.
From the FDA Drug Label
The FDA drug label does not answer the question.
From the Research
Typical Presentation of Spleen Lymphoma
- The most common presenting symptoms of spleen lymphoma are fever, malaise, and weight loss, as reported in a study of 20 patients with malignant lymphoma with primary presentation in the spleen 2.
- Physical examination often reveals prominent splenomegaly without palpable lymphadenopathy, as noted in the same study 2.
- Other clinical situations that can lead to the diagnosis of splenic lymphoma include splenomegaly, lymphocytosis, autoimmune hemolytic anemia, cold agglutinin disease, or cryoglobulinemia 3.
Histologic Types of Spleen Lymphoma
- Small lymphocytic lymphoma is the most frequent histologic type, followed by large cell lymphoma and mixed cell lymphoma, as found in a study of 20 patients with malignant lymphoma with primary presentation in the spleen 2.
- Other histologic types include marginal-zone splenic lymphoma, which originates from a peculiar splenic B-cell structure separated by the mantle zone, and splenic lymphoma with circulating villous lymphocytes 4.
- The morphologic diversity of spleen lymphoma emphasizes the need for initial recognition and correct classification, as survival is best determined according to histologic type 5.
Treatment Options for Spleen Lymphoma
- Splenectomy is still considered the most effective therapy for all primary splenic lymphomas, as stated in a study published in 1994 4.
- The importance of accurate splenic imaging in lymphoma assessment is also discussed, which has changed in recent years due to advances in therapy resulting in improvements in overall prognosis for both advanced and relapsed disease 6.
- The prognosis in primary splenic lymphoma appears to be similar to that in nodal lymphoma, with disease spreading outside of the spleen and its hilar lymph nodes being the single most important factor associated with an unfavorable prognosis 2, 4.