Management of patients with SMZL depends on the disease status, the severity of the clinical presentation, and the presence of other comorbidities.
44,45) However, splenic lymphoma with circulating villous lymphocytes has also been considered to be largely synonymous with SMZL, and precise discrimination of these disorders is challenging.
46,47) It is important to once again note that cytoplasmic projections are not specific to a particular type of lymphoma, and, as described previously, they can be seen in SMZL, HCL, and HCL-v, as well as other low-grade B-cell neoplasms.
44) and IgG; they are less likely to express CD103 than HCL-v, and CD27 is not expressed as frequently as it is in SMZL.
46) The discrimination between SDRPL and SMZL may not be possible on the basis of only the bone marrow and blood findings (7); however, therapeutic strategies for SDRPL do not differ from those of SMZL, and splenectomy solely to resolve uncertainty in classification is not suggested.
Lymphoplasmacytic lymphoma (LPL) is often in the differential diagnosis with SMZL because SMZL may exhibit plasmacytic differentiation, and LPL may sometimes be associated with splenomegaly.
50) MYD88 mutations are infrequent in SMZL and other low-grade B-cell lymphomas, (51,52) and detection of a MYD88 mutation is a useful diagnostic adjunct in the appropriate clinical setting.
55,56) It has also been recognized that some cases of B-PLL are associated with antecedent SMZL, suggesting that many of the remaining cases in the B-PLL category represent transformed SLs.
This was considered positive for involvement by SMZL.
8) Unfortunately, well-defined treatment strategies in SMZL and other indolent lymphomas have not been described.
Select patients with SMZL have an aggressive course with death occurring within 1 year of diagnosis, despite surgical and chemotherapeutic intervention.
Patient selection appears critical in SMZL, since some patients have long-term survival, while others have early progression.