Splenic lymphoma with villous lymphocytes

A rare type of non-Hodgkin lymphoma

Splenic lymphoma with villous lymphocytes (SLVL) is a rare type of non-Hodgkin lymphoma that primarily affects the spleen and is characterized by the presence of abnormal lymphocytes with villous projections. This condition is considered a subtype of splenic marginal zone lymphoma (SMZL).

Pathophysiology

SLVL is a B-cell lymphoma, meaning it originates from B lymphocytes, a type of white blood cell involved in the immune response. The disease is characterized by the proliferation of these abnormal B-cells, which accumulate in the spleen, bone marrow, and sometimes in the peripheral blood. The villous lymphocytes are so named because of their distinctive hair-like projections seen under a microscope.

Clinical Presentation

Patients with SLVL often present with an enlarged spleen, known as splenomegaly, which can cause discomfort or pain in the upper left abdomen. Other symptoms may include fatigue, weight loss, and night sweats. Some patients may also experience anemia, thrombocytopenia, or leukopenia due to the infiltration of the bone marrow by lymphoma cells.

Diagnosis

The diagnosis of SLVL is typically made through a combination of clinical evaluation, imaging studies, and laboratory tests. A complete blood count (CBC) may reveal abnormal lymphocytes in the blood. A bone marrow biopsy and immunophenotyping are often performed to confirm the diagnosis and to differentiate SLVL from other types of lymphomas. The presence of villous lymphocytes in the blood or bone marrow is a key diagnostic feature.

Treatment

The treatment of SLVL depends on the stage of the disease and the symptoms presented by the patient. In some cases, a "watch and wait" approach is adopted if the patient is asymptomatic. When treatment is necessary, options may include chemotherapy, immunotherapy, or splenectomy (surgical removal of the spleen). Rituximab, a monoclonal antibody targeting the CD20 antigen on B-cells, is commonly used in the treatment of SLVL.

Prognosis

The prognosis for patients with SLVL varies depending on several factors, including the stage of the disease at diagnosis and the patient's overall health. Generally, SLVL is considered an indolent lymphoma, meaning it progresses slowly. However, it can transform into a more aggressive form of lymphoma in some cases.

Related pages

• Non-Hodgkin lymphoma
• Splenic marginal zone lymphoma
• B-cell lymphoma
• Splenomegaly