TdT-positive high-grade B-cell lymphoma with BCOR and IDH1 mutations

Article Pathology

Blastoid high-grade B-cell lymphoma initially presenting in bone marrow: a diagnostic challenge

Mahsa Khanlari et al.

Summary: The study demonstrated significant differences in immunophenotypic, cytogenetic, and molecular features between blastoid-HGBL and B-ALL. The scoring system developed based on distinctive features showed high sensitivity and specificity for the classification of blastoid lymphoid tumors presenting initially in the bone marrow.

MODERN PATHOLOGY (2022)

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Article Pathology

Mutational Landscape of TdT+ Large B-cell Lymphomas Supports Their Distinction From B-lymphoblastic Neoplasms A Multiparameter Study of a Rare and Aggressive Entity

Shweta Bhavsar et al.

Summary: In the current classification of the World Health Organization, TdT expression in high grade/large cell B-cell lymphoma indicates B-lymphoblastic lymphoma/leukemia. However, there are reports of TdT expression in mature LBCL or following mature B-cell neoplasms. The frequency, categorization, clinicopathologic features, molecular landscape, and relationship to classic B-LBL of TdT(+) LBCL remain to be better defined. In this study, TdT expression was assessed in 258 LBCL and correlated with cytologic, phenotypic, and cytogenetic findings. Mutational analysis, review of prior biopsies, and assessment of clinical associations were performed in 6 cases with >10% TdT(+) cells. The 6 TdT(+) LBCL cases were blastoid-appearing, CD34(-), MYC+, BCL2(+), and had MYC rearrangements (R) (5/6 with BCL2 and/or BCL6-R). They had a prior TdT(-) LBCL and/or follicular lymphoma and all had an aggressive course. The mutational profiles were more like those reported in germinal center B-cell type-diffuse LBCL rather than B-LBL. The clinicopathologic and cytogenetic features of these 6 cases were similar to those found in a meta-analysis that included additional cases of TdT(+) LBCL or B-LBL following follicular lymphoma. Thus, TdT(+), CD34(-) large B-cell neoplasms with MYC rearrangements and often a double hit are rare, frequently a transformational event and aggressive but are distinct from classic B-LBL.

AMERICAN JOURNAL OF SURGICAL PATHOLOGY (2022)

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Article Hematology

The International Consensus Classification of Mature Lymphoid Neoplasms: a report from the Clinical Advisory Committee

Elias Campo et al.

Summary: Since 1994, the classification of lymphoid neoplasms has been continuously updated through international efforts, with recent progress driven by genomic studies. This proposal presents the International Consensus Classification of mature lymphoid, histiocytic, and dendritic cell tumors, which has refined diagnostic criteria and incorporated new insights from genomics.

BLOOD (2022)

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Review Oncology

The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Lymphoid Neoplasms

Rita Alaggio et al.

Summary: This article provides an overview of the upcoming 5th edition of the World Health Organization classification of lymphoid tumors, highlighting changes from the previous edition such as reorganization of entities, modification of nomenclature, revision of diagnostic criteria, deletion of entities, introduction of new entities, and inclusion of tumor-like lesions.

LEUKEMIA (2022)

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Review Hematology

BCOR gene alterations in hematologic diseases

Paolo Sportoletti et al.

Summary: The BCOR and BCORL1 genes have been found to have somatic clonal mutations in various hematologic malignancies and aplastic anemia. These mutations result in loss of the BCOR protein, consistent with its tumor suppressor role. Mouse models are important for understanding the role of Bcor loss in promoting hematologic malignancies in combination with alterations of other genes.

BLOOD (2021)

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Article Pathology