CD5-Negative, CD10-Negative Low-Grade B-Cell Lymphoproliferative Disorders of the Spleen

Abstract

CD5-negative, CD10-negative low-grade B-cell lymphoproliferative disorders (CD5-CD10-LPD) of the spleen comprise a fascinating group of indolent, neoplastic, mature B-cell proliferations that are essential to accurately identify but can be difficult to diagnose. They comprise the majority of B-cell LPDs primary to the spleen, commonly presenting with splenomegaly and co-involvement of peripheral blood and bone marrow, but with little to no involvement of lymph nodes. Splenic marginal zone lymphoma is one of the prototypical, best studied, and most frequently encountered CD5-CD10-LPD of the spleen and typically involves white pulp. In contrast, hairy cell leukemia, another well-studied CD5-CD10-LPD of the spleen, involves red pulp, as do the two less common entities comprising so-called splenic B-cell lymphoma/leukemia unclassifiable: splenic diffuse red pulp small B-cell lymphoma and hairy cell leukemia variant. Although not always encountered in the spleen, lymphoplasmacytic lymphoma, a B-cell lymphoproliferative disorder consisting of a dual population of both clonal B-cells and plasma cells and the frequent presence of the MYD88 L265P mutation, is another CD5-CD10-LPD that can be seen in the spleen. Distinction of these different entities is possible through careful evaluation of morphologic, immunophenotypic, cytogenetic, and molecular features, as well as peripheral blood and bone marrow specimens. A firm understanding of this group of low-grade B-cell lymphoproliferative disorders is necessary for accurate diagnosis leading to optimal patient management.

Elective Laparoscopic Splenectomy for Giant Cyst: Differential Diagnostics Issues, A Case Report

“…CD5negative, CD10-negative low-grade B-cell lymphoproliferative disorders of the spleen comprise a group of indolent, neoplastic, mature B-cell proliferations that are difficult to diagnose, due to due to exclusively multifaceted splenic localization (Figure 3). Central localization or increase in size of splenic cysts represents an indication for total splenectomy [10,14]. In Figure 4 we report computed tomographic images of splenic lesions of various nature.…”

Introduction to a Special Issue on Low-Grade B Cell Lymphoma in the Spleen

“…Biologically, any lymphoid cell lineage may constitute the cell of origin. However, low grade lymphomas derived from mature B cells, account for the vast majority of incurable cases, and are therefore the subject of this series of articles [ 1 , 2 , 3 , 4 , 5 ].…”

“…Using a practical binary classification of mature B cell lymphomas anchored on expression of CD5 and CD10, Schmieg et al illuminate the extensive topic of CD5/CD10-double-negative lymphoproliferative disorders [ 4 ]. These neoplasms traditionally include splenic marginal zone B cell lymphoma (SMZL), lymphoplasmacytic lymphoma, hairy cell leukemia and splenic B cell lymphoma/leukemia, unclassifiable (SBCLLU).…”