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How to Diagnose and Treat CD5-Positive Lymphomas Involving the Spleen
Review

Low-Grade Primary Splenic CD10-Positive Small B-Cell Lymphoma/Follicular Lymphoma

1
Department of Pathology, George Washington University, Washington, DC 20037, USA
2
City of Hope Medical Canter, Department of Pathology, Duarte, CA 91010, USA
3
Veterans Affairs Medical Center, Washington, DC 20052, USA
4
Department of Hematology, Initituto Português de Oncologia, 1649-028 Lisboa, Portugal
5
Veterans Affairs Medical Center, Department of Hematology, Washington, DC 20052, USA
*
Author to whom correspondence should be addressed.
Curr. Oncol. 2021, 28(6), 4821-4831; https://doi.org/10.3390/curroncol28060407
Received: 16 October 2021 / Revised: 13 November 2021 / Accepted: 15 November 2021 / Published: 18 November 2021
(This article belongs to the Special Issue B Cell Lymphoma in the Spleen)
Primary splenic lymphoma (PSL) is a rare malignancy representing about 1% of all lymphoproliferative disorders, when using a strict definition that allows only involvement of spleen and hilar lymph nodes. In contrast, secondary low-grade B-cell lymphomas in the spleen, such as follicular lymphomas (FL), lymphoplasmacytic lymphoma and chronic lymphocytic leukemia/ small lymphocytic lymphoma, particularly as part of advanced stage disease, are more common. Indolent B cell lymphomas expressing CD10 almost always represent FL, which in its primary splenic form is the focus of this review. Primary splenic follicular lymphoma (PSFL) is exceedingly infrequent. This type of lymphoproliferative disorder is understudied and, in most cases, clinically characterized by splenomegaly or cytopenias related to hypersplenism. The diagnosis requires correlation of histopathology of spleen, blood and/or bone marrow with the correct immunophenotype (determined by flow cytometry and/or immunohistochemistry) and if necessary, additional molecular profiling. Management of this incurable disease is evolving, and splenectomy remains the mainstream treatment for stage I PSFL. View Full-Text
Keywords: low-grade B-cell lymphomas; follicular lymphoma; primary splenic follicular lymphoma low-grade B-cell lymphomas; follicular lymphoma; primary splenic follicular lymphoma
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MDPI and ACS Style

Abdulbaki, R.; Tizro, P.; Nava, V.E.; Gomes da Silva, M.; Ascensão, J.L. Low-Grade Primary Splenic CD10-Positive Small B-Cell Lymphoma/Follicular Lymphoma. Curr. Oncol. 2021, 28, 4821-4831. https://doi.org/10.3390/curroncol28060407

AMA Style

Abdulbaki R, Tizro P, Nava VE, Gomes da Silva M, Ascensão JL. Low-Grade Primary Splenic CD10-Positive Small B-Cell Lymphoma/Follicular Lymphoma. Current Oncology. 2021; 28(6):4821-4831. https://doi.org/10.3390/curroncol28060407

Chicago/Turabian Style

Abdulbaki, Rami, Parastou Tizro, Victor E. Nava, Maria Gomes da Silva, and João L. Ascensão 2021. "Low-Grade Primary Splenic CD10-Positive Small B-Cell Lymphoma/Follicular Lymphoma" Current Oncology 28, no. 6: 4821-4831. https://doi.org/10.3390/curroncol28060407

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