Nodal marginal zone B cell lymphoma

Nodal marginal zone B cell lymphoma (NMZL) is a rare type of non-Hodgkin lymphoma that originates from B cells in the marginal zone of lymph nodes. It is characterized by slow growth and typically presents with lymphadenopathy, or swollen lymph nodes.

Epidemiology

NMZL is a rare disease, accounting for less than 2% of all non-Hodgkin lymphomas. It is more common in adults, with a median age of diagnosis in the sixth decade of life. There is no known gender predilection.

Pathogenesis

The exact cause of NMZL is unknown. However, it is believed to originate from B cells in the marginal zone of lymph nodes. These cells undergo malignant transformation, leading to the development of lymphoma. Some studies suggest a possible role of chronic inflammation or autoimmune disorders in the pathogenesis of NMZL.

Clinical Features

Patients with NMZL typically present with painless lymphadenopathy. Other symptoms may include fever, night sweats, and weight loss, collectively known as B symptoms. Some patients may also have splenomegaly, or an enlarged spleen.

Diagnosis

The diagnosis of NMZL is based on the histological examination of a lymph node biopsy. The lymphoma cells typically have a monocytoid appearance and are located in the marginal zone of the lymph node. Immunohistochemistry shows that the cells express B cell markers such as CD20 and CD79a.

Treatment

The treatment of NMZL depends on the stage and symptoms of the disease. For patients with localized disease, radiotherapy may be used. For those with advanced disease, chemotherapy with or without immunotherapy is typically recommended. The most commonly used chemotherapy regimen is R-CHOP, which includes rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone.

Prognosis

The prognosis of NMZL is generally good, with a 5-year survival rate of over 70%. However, the disease often has a relapsing-remitting course, and long-term follow-up is necessary.