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Review
. 2021 Nov 18;28(6):4821-4831.
doi: 10.3390/curroncol28060407.

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

Rami Abdulbaki  1 , Parastou Tizro  2 , Victor E Nava  1   3 , Maria Gomes da Silva  4 , João L Ascensão  5
Affiliations
Review

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

Rami Abdulbaki et al. Curr Oncol. .

Abstract

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.

Keywords: follicular lymphoma; low-grade B-cell lymphomas; primary splenic follicular lymphoma.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
(A,B) PSFL in the white pulp of the spleen (H&E, 40× and 100×, respectively). (C) Interphase between nodular white pulp with lymphomatous involvement and red pulp without lymphoma (H&E, 200×), (D) Lymphoma cells composed mainly of centrocytes (H&E, 400×).
Figure 1
Figure 1
(A,B) PSFL in the white pulp of the spleen (H&E, 40× and 100×, respectively). (C) Interphase between nodular white pulp with lymphomatous involvement and red pulp without lymphoma (H&E, 200×), (D) Lymphoma cells composed mainly of centrocytes (H&E, 400×).
Figure 2
Figure 2
Immunohistochemistry showing FL cells strongly positive for CD20 (A), CD10 (B), BCL6 (C) and BCL2 (D) (All at 400×).
See this image and copyright information in PMC

References

    1. Maguer-Satta V., Besançon R., Bachelard-Cascales E. Concise review: Neutral endopeptidase (CD10): A multifaceted environment actor in stem cells, physiological mechanisms, and cancer. Stem Cells. 2011;29:389–396. doi: 10.1002/stem.592. - DOI - PubMed
    1. Erdös E.G., Skidgel R.A. Neutral endopeptidase 24.11 (enkephalinase) and related regulators of peptide hormones. FASEB J. 1989;3:145–151. doi: 10.1096/fasebj.3.2.2521610. - DOI - PubMed
    1. McIntosh G.G., Lodge A.J., Watson P., Hall A.G., Wood K., Anderson J.J., Angus B., Horne C.H.W., Milton I.D. NCL-CD10-270: A new monoclonal antibody recognizing CD10 in paraffin-embedded tissue. Am. J. Pathol. 1999;154:77–82. doi: 10.1016/S0002-9440(10)65253-4. - DOI - PMC - PubMed
    1. Williamson J.M.S., Grigor I., Smith M.E.F., Holgate C.S., Quirke P., Bird C.C., Alison D.L., Child J.A. Cluster differentiation antigen expression, proliferative activity and clinical stage in centroblastic centrocytic lymphomas. J. Pathol. 1986;150:51–59. doi: 10.1002/path.1711500109. - DOI - PubMed
    1. Choi W.W.L., Weisenburger D.D., Greiner T.C., Piris M.A., Banham A.H., Delabie J., Braziel R.M., Geng H., Iqbal J., Lenz G., et al. A new immunostain algorithm classifies diffuse large B-cell lymphoma into molecular subtypes with high accuracy. Clin. Cancer Res. 2009;15:5494–5502. doi: 10.1158/1078-0432.CCR-09-0113. - DOI - PMC - PubMed

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