Circulating villous lymphocytes that mimics CLL, has an indolent course, associated with monoclonal protein spikes and has plasmacytoid basophilic lymphocytes that express CD11c.
Accounts for 0.6% of all non-Hodgkin’s lymphomas and <2% of all lymphoid malignancies.
Considered a low-grade B lymphoma with an indolent course.
Rarely causes B symptoms.
Most patients are asymptomatic and may not require treatment for many years.
Median survival exceeds 10 years.
Typically present with an enlarged spleen and involvement of splenic hilar lymph nodes and bone marrow.
Extrahilar lymphadenopathy is uncommon.
In one third of patients an aggressive course occurs with death within 4 years.
Patients present primarily with splenomegaly, and hepatomegaly can also be present but lymphadenopathy is rare.
Bone marrow involvement is invariable, with intrasunusoidal infiltration characteristic.
Patients with aggressive disease likely to have 7q31 deletion or lack IgVH somatic mutations, or have both abnormalities.
Prognosis with elevated beta-2 micro globulin, elevated white count of greater than 20,000 millimeter cube, and lymphocyte count grade and then 9000 µL have shorter survival times.
Overall survival less in patients with hemoglobin less than 12 g/dL, LDH greater than normal, and albumen less than 3.5 g/dL.
Patients with no risk factors are low-risk and have a five-year case specific survival of 88%.
In patients with one risk factor, an intermediate risk group, the case specific survival decreases to 73%, and the case specific survival decreases to 50% for those patients at high risk defined as having two or more risk factors
Many patients are asymptomatic and can be monitored for years prior to you and initiation of therapy.
All patients should be tested for hepatitis C, because successful antiviral therapy can improve response rate to the lymphoma.
Associated with del(7q31) and del (8P).
Indolent peripheral B cell neoplasm.
The lack of CD5 distinguishes SMZL from CLL and mantle cell lymphoma.
The lack of CD103 and CD25 distinguishes SMZL from hairy cell leukemia.
The immunophenotype of nodal marginal zone lymphoma is similar to that of splenic marginal zone lymphoma: clinical features assist with distinguishing between the two.
Splenic marginal zone lymphoma expresses B cell antigens CD 19, CD 20, CD 22, and it is typically CD5 negative, CD 10 negative, CD 43 negative, CD 103 negative and CV 25 negative.
Associated with chronic viral diseases such as hepatitis C and immune disorders.
Approximately 80% of patients have chromosomal ab2242ations.
The most frequent ab2242ations are gains of 3q,20-30% of cases, and 12q, 15-20% of cases, and deletion of 7q32, 30-40% of cases.
Median age greater than 60 years.
Slightly more common in women.
Typically associated with marked splenomegaly.
Treatment includes splenectomy, chemotherapy and Rituximab.
In refractory cases idealisib, ibrutinib and vorinostat should be considered, especially win combination with rituximab.