How we treat mature B-cell neoplasms (indolent B-cell lymphomas)

Lumish, Melissa; Falchi, Lorenzo; Imber, Brandon S.; Scordo, Michael; Keudell, Gottfried von; Joffe, Erel

doi:10.1186/s13045-020-01018-6

Cited by 22 publications

(19 citation statements)

References 240 publications

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“…SMZL patients that also have HCV infection may be treated with interferon-γ or ribavirin. Low dose radiation is usually only a second-line therapy for those with splenomegaly who failed other treatments [ 32 ]. A small portion (approximately 15%) of SMZL patients may have more aggressive disease.…”

Section: Splenic Marginal Zone Lymphomamentioning

confidence: 99%

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

Schmieg¹,

Muir²,

Aguilera

et al. 2021

Current Oncology

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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.

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Section: Splenic Marginal Zone Lymphomamentioning

confidence: 99%

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

Schmieg¹,

Muir²,

Aguilera

et al. 2021

Current Oncology

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“…Approximately 70% of patients have a progressive disease requiring treatment. Standard treatment options include splenectomy, Rituximab or Rituximab and bendamustine, all of which achieve high response rates with prolonged progression-free survival [8,9] . However, up to 30% of patients relapse and are intolerant of, or resistant to, standard therapies or transform to diffuse large B-cell lymphoma (DLBCL) with dismal survival [10] .…”

Section: Introductionmentioning

confidence: 99%

The (epi)genomic landscape of splenic marginal zone lymphoma, biological implications, clinical utility, and future questions

Oquendo¹,

Parker²,

Oscier³

et al. 2021

jtgg

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Splenic marginal zone lymphoma (SMZL) is an indolent B-cell lymphoma comprising less than 2% of lymphoid neoplasms. Approximately 70% of patients have a progressive disease requiring treatment and up to 30% of patients relapse or transform to diffuse large B-cell lymphoma. Whilst research over the last decade has transformed our understanding of many B-cell tumours, it is only beginning to shed light on the molecular pathogenesis of SMZL. Expansive immunogenetic investigations have shown biases in the immunoglobulin gene repertoire with distinct patterns of somatic hypermutation, suggesting a pathogenic role for antigen selection. In parallel cytogenetic studies have found a number of recurrent chromosomal lesions, in particular a deletion of the long arm of chromosome 7, though causative genes have not been identified. Our understanding of the mutational landscape of SMZL is built on a limited number of index cases, but has highlighted recurrent mutations in KLF2, NOTCH2 and TP53, and a spectrum of genes that cluster within biological pathways of importance in B-cell differentiation. While preliminary DNA methylation profiling has shown epigenetically distinct patient sub-groups, including a group defined by elevated expression of polycomb repressor complex 2 components. This review will provide an overview of our current understanding of the molecular basis of SMZL, and how this information impacts patient outcomes. Furthermore, we will outline; (1) the knowledge gaps that still exist; (2) a potential future research direction; and (3) how a detailed molecular understanding of the disease will ultimately provide

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“…Usualmente se manifiesta como enfermedad localizada, con síntomas gastrointestinales, pérdida de peso o sangrado oculto. Los "síntomas B" son infrecuentes (menos del 5%), puede progresar a otros tejidos y en raras ocasiones a un linfoma B difuso de células grandes o amiloidosis [11]. El rol del 18 Estimado Editor: Mujer de 58 años, con historia de dispepsia y tabaquismo, consultó por 6 meses de dorsalgia mecánica, progresiva, con pérdida significativa de peso, escalofríos e hiporexia.…”

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“…La histología demuestra células pequeñas con folículos reactivos en la zona marginal y/o la región interfolicular. El inmunofenotipo corresponde a linfocitos B positivos para CD19, CD20, y CD22 y negativos para CD5, CD10 y CD23, frecuentemente asociados a anormalidades cromosómicas (trisomía 3 o t(11;18)) [9,11]. El tratamiento inicial depende del estadio, localización, y la presencia de H. pylori, pues su erradicación generalmente obtiene la regresión del linfoma.…”

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“…El tratamiento inicial depende del estadio, localización, y la presencia de H. pylori, pues su erradicación generalmente obtiene la regresión del linfoma. Este linfoma incrementa el riesgo de adenocarcinoma gástrico y de otros linfomas, por tanto, requiere seguimiento [11]. En nuestro caso, la clasificación corresponde a T1m N0 M0 (Paris staging system), I (Lugano staging system), y tiene indicado seguimiento para evaluar erradicación.…”

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