Introduction
Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue
lymphomas (MALT lymphoma) belongs to mature B-cell neoplasms and thus to
the huge class of non-Hodgkin’s lymphoma (NHL). MALT lymphoma account
for 7-8% of B-cell lymphomas, most commonly affecting the stomach
(35%), ocular adnexa (13%), skin (9%), lungs (9%), salivary glands
(8%), breasts (3%) and thyroid (2%).
The bronchus-associated lymphoid tissue lymphoma (BALT lymphoma)
represents the most common histologic subtype of primary pulmonary
lymphomas (PPL) with 77 – 87%. However, PPLs are rare with an
occurrence of 0.5 – 1% of all pulmonary tumors. Causes to develop a
BALT lymphoma are various and not completely understood. In many MALT
lymphomas, there is a history of chronic inflammatory disease resulting
in the accumulation of extranodal lymphoid tissue. The chronic
inflammation may be the result of infection, autoimmunity, smoking,
environmental or other unknown risk factors.
Patients are mostly asymptomatic at diagnosis and if symptoms are
present, they are rather nonspecific including dyspnea, cough, or
B-symptoms for several weeks or months. For diagnosis clinical, medical
imaging, histologic and biologic parameters are needed. BALT lymphomas
progress slowly and mostly remain within the lung for a long time.
Besides treatment/eradication of an underlying chronic inflammatory
disorder/trigger factor combined chemo- and/or immunotherapy, surgery
and/or local radiation are possible treatment approches. Currently, no
standard treatment approach is available partially due to its rare
occurrence and heterogeneity. Nevertheless, BALT lymphomas are
associated with a favorable prognosis and a 5-year overall survival (OS)
rate of 80% as well as median survival over 10 years.
Here, we report two patients with nonspecific symptoms and blood count
anomalies, who were diagnosed with BALT lymphoma in our hospital and
treated with a combined immune-chemotherapy of rituximab and
bendamustine.