New Insights into the Biology and Diagnosis of Splenic Marginal Zone Lymphomas

Abstract

Splenic marginal zone lymphoma (SMZL) is a small B-cell lymphoma, which has been recognized as a distinct pathological entity since the WHO 2008 classification. It classically presents an indolent evolution, but a third of patients progress rapidly and require aggressive treatments, such as immuno-chemotherapy or splenectomy, with all associated side effects. In recent years, advances in the comprehension of SMZL physiopathology have multiplied, thanks to the arrival of new devices in the panel of available molecular biology techniques, allowing the discovery of new molecular findings. In the era of targeted therapies, an update of current knowledge is needed to guide future researches, such as those on epigenetic modifications or the microenvironment of these lymphomas.

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

“…However, if a rare CD10-negative FL is considered in the differential diagnosis, additional markers such as IRTA and MNDA (which are commonly positive in MZL and negative in FL) may be necessary [ 45 ]. Other CD5/CD10-double negative low-grade B cell lymphomas may very infrequently show aberrant expression of CD10, posing a diagnostic challenge to differentiate from FL (particularly with splenic presentation), as covered extensively elsewhere and in this review series [ 42 , 43 , 44 , 45 , 46 ] (and cross referencing Auerbach, Gomes and Aggarwal’s papers). For example, HCL can express CD10 [ 7 ], but its triple positivity for CD25, CD103 and CD123 together with the absence of BCL6 expression (usually positive in FL), and the presence of BRAF V600E alteration, should permit clear segregation from other small B cell lymphomas, including PSFL [ 7 ].…”

Hairy Cell Leukemia and HCL-Like Disorders: Diagnosis and TreatmentLeuc閙ie �Tricholeucocytes et Autres Prolif閞ations �Cellules Chevelues: Diagnostic et Traitement

“…Le LSZM est une entité à part entière. Il existe dans les formes spléniques des lymphomes de la zone marginale (SMZL) des formes avec cellules circulantes, souvent en faible nombre (<20%) avec des villosités courtes et associées à un composant lymphoplasmocytaire [155]. La splénectomie, si elle était réalisée, montrerait une expansion de la pulpe blanche et une infiltration de la pulpe rouge.…”

Artificial Intelligence Predicted Overall Survival and Classified Mature B-Cell Neoplasms Based on Immuno-Oncology and Immune Checkpoint Panels

“…Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue is an extranodal lymphoma (MALT lymphoma) composed of a heterogeneous population of small B-cells [ 3 ]. It originates in the marginal zones, but it extends into the interfollicular and follicular regions and infiltrates the epithelium, forming the lymphoepithelial lesions [ 3 , 11 ].…”