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A Review on Splenic Diffuse Red Pulp Small B-Cell Lymphoma
| Content Provider | MDPI |
|---|---|
| Author | Yilmaz, Elif Chhina, Arashpreet Nava, Victor E. Aggarwal, Anita |
| Copyright Year | 2021 |
| Description | Splenic diffuse red pulp small B-cell lymphoma (SDRPL) is a rare disease, representing <1% of all non-Hodgkin lymphomas (NHL). The most common clinical manifestations include splenomegaly, lymphocytosis, and hemocytopenia. A diagnosis of SDRPL can be challenging, as it shares multiple clinical and laboratory features with splenic marginal zone lymphoma (SMZL), hairy cell leukemia (HCL), and HCL variant (HCL-v). Obtaining splenic tissue remains the gold standard for diagnosis. In the cases where splenic tissue is not available, diagnosis can be established by a review of peripheral blood and bone marrow studies. SDRPL is characterized by a diffuse involvement of the splenic red pulp by monomorphous small-to-medium sized mature B lymphocytes effacing the white pulp. The characteristic immunophenotype is positive for CD20, DBA.44 (20 to 90%), and IgG, and typically negative for CD5, CD10, CD23, cyclin D1, CD43, annexin A1, CD11c, CD25, CD123, and CD138. The Ki-67 proliferative index is characteristically low. Cyclin D3 is expressed in the majority of SDRPL in contrast with other types of small B-cell lymphomas, thus facilitating the recognition of this disease. There is no standard treatment regimen for SDRPL. Initial treatment options include splenectomy, rituximab monotherapy, or a combination of both. Chemoimmunotherapy should be considered in patients with advanced disease at baseline or progression. |
| Ending Page | 5154 |
| Page Count | 7 |
| Starting Page | 5148 |
| e-ISSN | 17187729 |
| DOI | 10.3390/curroncol28060431 |
| Journal | Current Oncology |
| Issue Number | 6 |
| Volume Number | 28 |
| Language | English |
| Publisher | MDPI |
| Publisher Date | 2021-12-06 |
| Access Restriction | Open |
| Subject Keyword | Current Oncology Hematology Pathology Primary Splenic Lymphoma Splenic Diffuse Red Pulp Lymphoma Villous Lymphocytes B-cell Lymphoma Non-hodgkin Lymphoma |
| Content Type | Text |
| Resource Type | Article |
