Splenic red pulp lymphoma with numerous basophilic villous lymphocytes: a distinct clinicopathologic and molecular entity?

Traverse-Gléhen, Alexandra; Baseggio, Lucile; Bauchu, Evelyne Callet; Morel, Dominique; Gazzo, Sophie; Ffrench, Martine; Verney, Aurélie; Rolland, Delphine C.M.; Thiéblemont, Catherine; Magaud, Jean‐Pierre; Salles, Gilles; Coiffier, Bertrand; Berger, Françoise; Felman, Pascale

doi:10.1182/blood-2007-07-098848

Cited by 149 publications

(143 citation statements)

References 33 publications

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“…3 Noticeably, we found a marked under-representation of the IGHV1-2 gene among cases with spleen histopathology suggestive of SDRL, in keeping with our previous report. 4 On these grounds, we propose that immunogenetic analysis may add discriminative power in the differential diagnosis between SMZL and other primary splenic small B-cell lymphomas of marginal-zone origin assigned by the 2008 WHO classification to the provisional category of SLLU.…”

Section: Discussionmentioning

confidence: 95%

“…More recently, it became clear that SMZL shows distinct characteristics compared with other low-grade B-cell lymphomas, enabling its inclusion in the 2008 WHO classification of Tumors of the Haematopoietic and Lymphoid tissues as a distinct clinical and pathological entity. 3 In addition to SMZL, the 2008 WHO classification recognizes a provisional category of splenic lymphoma/leukemia unclassifiable (SLLU), including entities such as splenic diffuse red pulp lymphoma (SDRL) 4 and hairy cell leukemia-variant. 5 Currently, the ontogenetic relationship of SMZL to these provisional entities remains undefined.…”

Section: Introductionmentioning

confidence: 99%

“…7,8 Previous studies by us and others have shown that SMZL shows a biased IG gene repertoire and is characterized by heterogeneity with regard to the mutational status of the IG receptors. 4,9 --23 Individual studies have highlighted different IG sequence features, including repertoire differences between SMZL, SDRL and hairy cell leukemia-variant; 4,15 intraclonal diversification of IG genes through ongoing SHM; 12,17,18,23 potential analogies between SMZL and hairy cell leukemia-variant; 15 and, very recently, the existence of cases sharing quasi-identical antigen-binding sites (stereotyped B-cell receptors). 21 Furthermore, some studies have also examined whether particular molecular characteristics of the IG receptors might be associated with genetic or phenotypic features, and/or clinical outcome.…”

Section: Introductionmentioning

confidence: 99%

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Over 30% of patients with splenic marginal zone lymphoma express the same immunoglobulin heavy variable gene: ontogenetic implications

et al. 2012

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We performed an immunogenetic analysis of 345 IGHV --IGHD --IGHJ rearrangements from 337 cases with primary splenic small B-cell lymphomas of marginal-zone origin. Three immunoglobulin (IG) heavy variable (IGHV) genes accounted for 45.8% of the cases (IGHV1-2, 24.9%; 12.8%; 8.1%). Particularly for the IGHV1-2 gene, strong biases were evident regarding utilization of different alleles, with 79/86 rearrangements (92%) using allele *04. Among cases more stringently classified as splenic marginal-zone lymphoma (SMZL) thanks to the availability of splenic histopathological specimens, the frequency of IGHV1-2*04 peaked at 31%. The IGHV1-2*04 rearrangements carried significantly longer complementaritydetermining region-3 (CDR3) than all other cases and showed biased IGHD gene usage, leading to CDR3s with common motifs. The great majority of analyzed rearrangements (299/345, 86.7%) carried IGHV genes with some impact of somatic hypermutation, from minimal to pronounced. Noticeably, 75/79 (95%) IGHV1-2*04 rearrangements were mutated; however, they mostly (56/75 cases; 74.6%) carried few mutations (97 --99.9% germline identity) of conservative nature and restricted distribution. These distinctive features of the IG receptors indicate selection by (super)antigenic element(s) in the pathogenesis of SMZL. Furthermore, they raise the possibility that certain SMZL subtypes could derive from progenitor populations adapted to particular antigenic challenges through selection of VH domain specificities, in particular the IGHV1-2*04 allele.

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Section: Discussionmentioning

confidence: 95%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Over 30% of patients with splenic marginal zone lymphoma express the same immunoglobulin heavy variable gene: ontogenetic implications

et al. 2012

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“…This case displayed a remarkable red pulp growth pattern in the spleen, suggesting a possible diagnosis of diffuse red pulp small B-cell lymphoma, a provisional entity in the 2008 WHO classification where the cells are described as having plasmacytoid features. [33][34][35] Subsequent examination of earlier bone marrow biopsies, however, showed no sign of the intrasinusoidal growth pattern characteristic of this entity, but rather a diffuse lymphoplasmacytic infiltrate that would be consistent with a diagnosis of lymphoplasmacytic lymphoma. Although there are little data in the recent literature regarding the splenic histology of lymphoplasmacytic lymphoma as defined by 2008 WHO criteria, the older literature does describe cases of putative lymphoplasmacytic lymphoma with a predominantly red pulp pattern of involvement.…”

Section: F Hamadeh Et Almentioning

confidence: 99%

MYD88 L265P mutation analysis helps define nodal lymphoplasmacytic lymphoma

et al. 2015

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The diagnosis of lymphoplasmacytic lymphoma is often challenging, especially in extramedullary tissues where the differential diagnosis includes nodal marginal zone lymphoma, splenic marginal zone lymphoma, or other small B-cell neoplasms with plasmacytic differentiation. The MYD88 L265P mutation has been recently identified in 490% of bone-marrow-based lymphoplasmacytic lymphoma, but the incidence of this abnormality and corresponding morphologic correlates in nodal lymphoplasmacytic lymphoma have not been established. We analyzed 87 cases of extramedullary lymphoplasmacytic lymphoma, splenic marginal zone lymphoma, unclassifiable splenic B-cell lymphomas, nodal marginal zone lymphoma with plasmacytic differentiation, and chronic lymphocytic leukemia/small lymphocytic lymphoma with plasmacytic differentiation for MYD88 L265P. Eighteen cases (21%) were positive, including 9/9 (100%) lymphoplasmacytic lymphomas with classic histologic features, 5/12 (42%) cases that met 2008 WHO criteria for lymphoplasmacytic lymphoma but with atypical morphologic features, 3/15 (20%) cases initially considered nodal marginal zone lymphoma with plasmacytic differentiation, and 1/6 (17%) unclassifiable splenic B-cell lymphomas. The presence of MYD88 L265P was associated with IgM paraprotein (Po0.001) and a trend for bone marrow involvement (P ¼ 0.09). Each of 44 splenectomy-defined splenic marginal zone lymphomas (19 with plasmacytic differentiation) and the chronic lymphocytic leukemia/small lymphocytic lymphoma with plasmacytic differentiation were negative for the mutation. Morphologic re-review with knowledge of MYD88 mutation status and all available clinical features suggested all MYD88 mutated cases were consistent with lymphoplasmacytic lymphoma (either classic or variant histology), except for one case which remained most consistent with nodal marginal zone lymphoma with plasmacytic differentiation. These results demonstrate the importance of MYD88 mutational analysis in better defining lymphoplasmacytic lymphoma as a relatively monomorphic small B-cell lymphoma with plasmacytic differentiation that may show total nodal architectural effacement and follicular colonization. Cases previously considered lymphoplasmacytic lymphoma that are more polymorphous and are often associated with histiocytes should no longer be included in the lymphoplasmacytic lymphoma category. Clinicopathologic review suggests that although MYD88 mutated non-lymphoplasmacytic lymphoma small B-cell neoplasms exist, they are very uncommon.

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“…HCL, which is four to five times more frequent in men than women, accounts for 2% of all leukemias with approximately 1000 new cases being reported in the United States each year. HCL must be differentiated from other HCL‐like disorders, including hairy cell leukemia variant (HCL‐V)3 and splenic diffuse red pulp lymphoma (SDRPL) 4. In this article, we review the significant advancements that have occurred over the last three years in the understanding of the pathobiology of HCL and HCL‐like disorders and provide an update on the new treatment procedures now available, particularly for patients with relapsed/refractory HCL.…”

Section: Introductionmentioning

confidence: 99%

Hairy cell leukemia 2018: Update on diagnosis, risk‐stratification, and treatment

Troussard

Cornet

2017

American J Hematol

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Disease overviewHairy cell leukemia (HCL) and HCL‐like disorders, including HCL variant (HCL‐V) and splenic diffuse red pulp lymphoma (SDRPL), are a very heterogeneous group of mature lymphoid B‐cell disorders, characterized by the identification of hairy cells, a specific genetic profile, a different clinical course and the need for appropriate treatment.DiagnosisDiagnosis of HCL is based on morphological evidence of hairy cells, an HCL immunologic score of 3 or 4 based on the CD11C, CD103, CD123, and CD25 expression, the trephine biopsy which makes it possible to specify the degree of tumoral medullary infiltration and the presence of BRAF V600E somatic mutation.Risk stratificationProgression of patients with HCL is based on a large splenomegaly, leukocytosis, a high number of hairy cells in the peripheral blood and the immunoglobulin heavy chain variable region gene mutational status. VH4‐34 positive HCL cases are associated with poor prognosisRisk adapted therapyPurine analogs (PNA) are indicated in symptomatic first line HCL patients. The use of PNA followed by rituximab represents an alternative option.Management of progressive or refractory diseaseIt is based on the use of BRAF inhibitors associated or not with MEK inhibitors, recombinant immunoconjugates targeting CD22 or BCR inhibitors.

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Splenic red pulp lymphoma with numerous basophilic villous lymphocytes: a distinct clinicopathologic and molecular entity?

Cited by 149 publications

References 33 publications

Over 30% of patients with splenic marginal zone lymphoma express the same immunoglobulin heavy variable gene: ontogenetic implications

Over 30% of patients with splenic marginal zone lymphoma express the same immunoglobulin heavy variable gene: ontogenetic implications

MYD88 L265P mutation analysis helps define nodal lymphoplasmacytic lymphoma

Hairy cell leukemia 2018: Update on diagnosis, risk‐stratification, and treatment

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