Splenic Lymphomas: A Tertiary Care Center Experience and Review of Literature

Abstract

Primary splenic lymphomas are rare with the majority of lymphomas in spleen being secondary to an extra-splenic lymphoma. We aimed to analyze the epidemiological profile of the splenic lymphoma and review the literature. This was a retrospective study including all splenectomies and splenic biopsies from 2015 to September 2021. All the cases were retrieved from Department of Pathology. Detailed histopathological, clinical and demographic evaluation was done. All the lymphomas were classified according to WHO 2016 classification. A total of 714 splenectomies were performed for a variety of benign causes, as part of tumor resections and for the diagnosis of lymphoma. Few core biopsies were also included. A total of 33 lymphomas diagnosed in the spleen, primary splenic lymphomas constituted 84.84% (n = 28) of the cohort with 5 (15.15%) having the primary site elsewhere. The primary splenic lymphomas constituted 0.28% of all the lymphomas arising at various sites. Adult population (19-65 years) formed the bulk (78.78%) with a slight male preponderance. Splenic marginal zone lymphomas (n = 15, 45.45%) comprised of major proportion of cases followed by primary splenic diffuse large B-cell lymphoma (n = 4, 12.12%). Splenectomy was the main course of treatment for SMZL with a good overall outcome, with chemotherapy ± radiotherapy forming the mainstay in other lymphomas. Lymphomas in spleen can be infiltrative or a primary, hence proper clinic-radiological and pathological evaluation is required. Appropriate management is guided by the precise and detailed evaluation by the pathologist, requiring understanding of the same.

Keywords: Primary splenic lymphomas; Splenectomy; Splenic marginal zone lymphomas.

Fig. 1

Preferential involvement of red [A] and white pulp [B] by different lymphomas DLBCL-Diffuse large B-cell lymphoma, PTCL- Peripheral T-cell lymphoma, CLL/SLL-Chronic lymphocytic leukemia/small lymphocytic lymphoma, AITL-Angioimmunoblastic T-cell lymphoma

Fig. 2

Distribution of Primary splenic lymphomas in our study; Splenic marginal zone lymphomas formed the majority of the study group

Fig. 3

Microphotograph of normal spleen showing white pulp and red pulp. [H&E Ax16]. Splenic marginal zone lymphoma: Microphotograph showing Expanded white pulp with prominent marginal zone. [B × 40, C × 100] There is coalescence of follicles(Red arrow) [C × 100]. Red pulp shows small lymphoid nodules [D × 100].The cells are immunopositive for CD20 [E], BCl2 [F], negative for CD23 [G]. Mantle cell lymphoma: Sheets of atypical lymphoid cells [H × 100] positive for CD20 [I] and Cyclin D1 [J]. Diffuse large B cell lymphoma: Needle core biopsy from spleen shows large atypical lymphoid cell involving red pulp. [K × 100]. The cells are immunopositive for CD20 [L]. Splenic diffuse red pulp lymphoma: Section shows marked expansion of the red pulp with infiltration by small size lymphoid cells. Small atretic white pulps are seen. [M × 16, N × 100]. The cells are immunopositive for CD20 [N]

Fig. 4

Anaplastic large cell lymphoma: Microphotograph shows needle core biopsy from Spleen with infiltration of red pulp by large atypical lymphoid cells with moderate amount of cytoplasm. [Ax40, Bx200]The cells are immunopositive for CD30 [C] and ALK [D]. Peripheral T-cell lymphoma, NOS: Section shows diffuse infiltration of red pulp by small to intermediate size atypical lymphoid cells which are immunopositive for CD3 [F], CD5 [G] with partial loss of CD7 [H]. Hepatosplenic gamma delta T-cell lymphoma: Microphotograph showing red pulp involvement by atypical large cells [I × 200]. Involvement of liver with sinusoidal infiltration by atypical lymphoid cells [J, × 200]. Atypical lymphoid cells immunopositive for CD3[K × 200] and CD56 [L × 200]

Fig. 5

Approach to lymphomas involving spleen with predominant white pulp involvement

Fig. 6

Approach to lymphomas involving spleen with predominant red pulp involvement