B Cell Lymphoma in the Spleen

A special issue of Current Oncology (ISSN 1718-7729). This special issue belongs to the section "Hematology".

Deadline for manuscript submissions: closed (10 October 2021) | Viewed by 24974

Special Issue Editor


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Guest Editor
1. Veterans Affairs Medical Center, Washington, DC 20422, USA
2. Department of Pathology, George Washington University, Washington, DC 20037, USA
Interests: pathology; hematopathology; molecular biology; virology; immunology; oncogenesis; parasitology
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Special Issue Information

Dear Colleagues,

Malignant lymphoma in the spleen may be primary (usually designated as splenic lymphoma) or secondary (due to progression of nodal or extra nodal lymphoproliferative disease), and represents an underestimated cause of splenomegaly, since splenectomy is seldom performed.  Lymphomatous involvement of the spleen represents widely heterogenous clinicopathologic features, ranging from indolent to highly aggressive disease, which still represent a therapeutic challenge.  Biologically, any hematopoietic cell lineage may represent the cell of origin. However, low grade B cell lymphomas probably represent the vast majority of incurable cases and are, therefore, the subject of this review series.

Through a series of papers, we will describe the most common entities based on the approach of the WHO classification of tumors integrating clinical, morphological, immunophenotypical, and molecular characteristics to review the current therapeutic modalities.

This Special Issue would include, but not be limited to, the following topics: CD5-positive B cell lymphoma, CD10-positive B cell lymphoma, CD5/CD10-double negative B cell lymphoma, and primary splenic lymphoma.

A practical emphasis on establishing a differential diagnosis and choosing the most appropriate therapy based on current molecular profiling will be made.

In summary, we will provide a state-of-the-art review on B cell lymphomas in the spleen that clinicians, pathologists, and basic science investigators may find informative.

Dr. Victor E. Nava
Guest Editor

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Published Papers (6 papers)

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Editorial

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2 pages, 167 KiB  
Editorial
Introduction to a Special Issue on Low-Grade B Cell Lymphoma in the Spleen
by Victor E. Nava
Curr. Oncol. 2022, 29(1), 130-131; https://doi.org/10.3390/curroncol29010011 - 28 Dec 2021
Viewed by 1388
Abstract
Malignant lymphoproliferative disorders in the spleen may be primary (usually designated as splenic lymphoma) or secondary (due to progression of nodal or extra nodal lymphoid neoplasms) and represent an underestimated cause of splenomegaly, partially due to the decreasing frequency of splenectomy in our [...] Read more.
Malignant lymphoproliferative disorders in the spleen may be primary (usually designated as splenic lymphoma) or secondary (due to progression of nodal or extra nodal lymphoid neoplasms) and represent an underestimated cause of splenomegaly, partially due to the decreasing frequency of splenectomy in our era of personalized molecular medicine [...] Full article
(This article belongs to the Special Issue B Cell Lymphoma in the Spleen)

Review

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24 pages, 4239 KiB  
Review
CD5-Negative, CD10-Negative Low-Grade B-Cell Lymphoproliferative Disorders of the Spleen
by John J. Schmieg, Jeannie M. Muir, Nadine S. Aguilera and Aaron Auerbach
Curr. Oncol. 2021, 28(6), 5124-5147; https://doi.org/10.3390/curroncol28060430 - 4 Dec 2021
Cited by 1 | Viewed by 4658
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, [...] Read more.
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. Full article
(This article belongs to the Special Issue B Cell Lymphoma in the Spleen)
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11 pages, 3145 KiB  
Review
Low-Grade Primary Splenic CD10-Positive Small B-Cell Lymphoma/Follicular Lymphoma
by Rami Abdulbaki, Parastou Tizro, Victor E. Nava, Maria Gomes da Silva and João L. Ascensão
Curr. Oncol. 2021, 28(6), 4821-4831; https://doi.org/10.3390/curroncol28060407 - 18 Nov 2021
Cited by 3 | Viewed by 2888
Abstract
Primary splenic lymphoma (PSL) is a rare malignancy representing about 1% of all lymphoproliferative disorders, when using a strict definition that allows only involvement of spleen and hilar lymph nodes. In contrast, secondary low-grade B-cell lymphomas in the spleen, such as follicular lymphomas [...] Read more.
Primary splenic lymphoma (PSL) is a rare malignancy representing about 1% of all lymphoproliferative disorders, when using a strict definition that allows only involvement of spleen and hilar lymph nodes. In contrast, secondary low-grade B-cell lymphomas in the spleen, such as follicular lymphomas (FL), lymphoplasmacytic lymphoma and chronic lymphocytic leukemia/ small lymphocytic lymphoma, particularly as part of advanced stage disease, are more common. Indolent B cell lymphomas expressing CD10 almost always represent FL, which in its primary splenic form is the focus of this review. Primary splenic follicular lymphoma (PSFL) is exceedingly infrequent. This type of lymphoproliferative disorder is understudied and, in most cases, clinically characterized by splenomegaly or cytopenias related to hypersplenism. The diagnosis requires correlation of histopathology of spleen, blood and/or bone marrow with the correct immunophenotype (determined by flow cytometry and/or immunohistochemistry) and if necessary, additional molecular profiling. Management of this incurable disease is evolving, and splenectomy remains the mainstream treatment for stage I PSFL. Full article
(This article belongs to the Special Issue B Cell Lymphoma in the Spleen)
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23 pages, 1435 KiB  
Review
How to Diagnose and Treat CD5-Positive Lymphomas Involving the Spleen
by José Cabeçadas, Victor E. Nava, Joao L. Ascensao and Maria Gomes da Silva
Curr. Oncol. 2021, 28(6), 4611-4633; https://doi.org/10.3390/curroncol28060390 - 11 Nov 2021
Cited by 4 | Viewed by 4785
Abstract
Patients with CD5-expressing lymphomas presenting with splenomegaly are frequently diagnosed with chronic lymphocytic leukemia. The most important differential diagnosis is mantle cell lymphoma, both in its classical and leukemic, non-nodal forms, given its prognostic and therapeutic implications. Other small B-cell neoplasms that frequently [...] Read more.
Patients with CD5-expressing lymphomas presenting with splenomegaly are frequently diagnosed with chronic lymphocytic leukemia. The most important differential diagnosis is mantle cell lymphoma, both in its classical and leukemic, non-nodal forms, given its prognostic and therapeutic implications. Other small B-cell neoplasms that frequently involve the spleen and occasionally express CD5 include the splenic marginal zone lymphoma, hairy cell leukemia and, rarely, lymphoplasmacytic lymphoma. The frequency of CD5 positivity depends in part on the sensitivity of the detection methods employed. Usually, a combination of morphological, immunophenotypic and molecular findings allows for a precise sub-classification of CD5-positive, low-grade B-cell lymphomas of the spleen. Some of these tumors may display a mixture of small and larger B cells, raising the possibility of more aggressive lymphomas, such as diffuse large B-cell lymphomas (DLBCL). Approximately 5–10% of DLBCL are CD5-positive and some may manifest as primary splenic lesions. When available, the morphology of DLBCL in the splenic tissue is distinctive and a leukemic picture is very rare. In conclusion, the appropriate morphological and clinical context assisted by flow cytometry panels and/or immunohistochemistry allows the differential diagnosis of CD5-positive, non-Hodgkin, B-cell lymphomas involving the spleen. Full article
(This article belongs to the Special Issue B Cell Lymphoma in the Spleen)
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18 pages, 5427 KiB  
Review
New Insights into the Biology and Diagnosis of Splenic Marginal Zone Lymphomas
by Marie Donzel, Lucile Baseggio, Juliette Fontaine, Florian Pesce, Hervé Ghesquières, Emmanuel Bachy, Aurélie Verney and Alexandra Traverse-Glehen
Curr. Oncol. 2021, 28(5), 3430-3447; https://doi.org/10.3390/curroncol28050297 - 6 Sep 2021
Cited by 9 | Viewed by 6039
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 [...] Read more.
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. Full article
(This article belongs to the Special Issue B Cell Lymphoma in the Spleen)
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Other

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7 pages, 2168 KiB  
Perspective
A Review on Splenic Diffuse Red Pulp Small B-Cell Lymphoma
by Elif Yilmaz, Arashpreet Chhina, Victor E. Nava and Anita Aggarwal
Curr. Oncol. 2021, 28(6), 5148-5154; https://doi.org/10.3390/curroncol28060431 - 6 Dec 2021
Cited by 15 | Viewed by 4224
Abstract
Splenic diffuse red pulp small B-cell lymphoma (SDRPL) is a rare disease, representing <1% of all non-Hodgkin lymphomas (NHL). The most common clinical manifestations include splenomegaly, lymphocytosis, and hemocytopenia. A diagnosis of SDRPL can be challenging, as it shares multiple clinical and laboratory [...] Read more.
Splenic diffuse red pulp small B-cell lymphoma (SDRPL) is a rare disease, representing <1% of all non-Hodgkin lymphomas (NHL). The most common clinical manifestations include splenomegaly, lymphocytosis, and hemocytopenia. A diagnosis of SDRPL can be challenging, as it shares multiple clinical and laboratory features with splenic marginal zone lymphoma (SMZL), hairy cell leukemia (HCL), and HCL variant (HCL-v). Obtaining splenic tissue remains the gold standard for diagnosis. In the cases where splenic tissue is not available, diagnosis can be established by a review of peripheral blood and bone marrow studies. SDRPL is characterized by a diffuse involvement of the splenic red pulp by monomorphous small-to-medium sized mature B lymphocytes effacing the white pulp. The characteristic immunophenotype is positive for CD20, DBA.44 (20 to 90%), and IgG, and typically negative for CD5, CD10, CD23, cyclin D1, CD43, annexin A1, CD11c, CD25, CD123, and CD138. The Ki-67 proliferative index is characteristically low. Cyclin D3 is expressed in the majority of SDRPL in contrast with other types of small B-cell lymphomas, thus facilitating the recognition of this disease. There is no standard treatment regimen for SDRPL. Initial treatment options include splenectomy, rituximab monotherapy, or a combination of both. Chemoimmunotherapy should be considered in patients with advanced disease at baseline or progression. Full article
(This article belongs to the Special Issue B Cell Lymphoma in the Spleen)
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