ArticleModern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc2013
ALK-positive large B-cell lymphomas express a terminal B-cell differentiation program and activated STAT3 but lack MYC rearrangements.
Article in Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers.
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18 citing papers in PubMed, 65 citations in OpenAlex.
- Anaplastic Lymphoma Kinase (ALK)-Positive Large B-cell Lymphoma in Children: A Case Report and Review of Literature.Cureus · 2026Article
- CD20-Negative Large B-Cell Lymphomas: The Diagnostic Challenge of Tumors with Downregulation of Mature B-Cell Marker Expression.International journal of molecular sciences · 2025Review
- Review
- Primary anaplastic lymphoma kinase-positive large B-cell lymphoma of the left bulbar conjunctiva: A case report.World journal of clinical cases · 2024Article
- Review
- Plasmablastic lymphoma occurring in ulcerative colitis during treatment with immunosuppressive therapy.Clinical journal of gastroenterology · 2023Article
- MAPK and JAK-STAT pathways dysregulation in plasmablastic lymphoma.Haematologica · 2021Article
- Genetic lesions in MYC and STAT3 drive oncogenic transcription factor overexpression in plasmablastic lymphoma.Haematologica · 2021Article
- Genomic characterization of HIV-associated plasmablastic lymphoma identifies pervasive mutations in the JAK-STAT pathway.Blood cancer discovery · 2020Article
- Plasmablastic Lymphoma Associated with Adjacent Mature Plasma Cell Population Exhibiting Opposite Light Chain Restriction.Case reports in pathology · 2020Article
- Diagnosis and classification of lymphoma: Impact of technical advances.Seminars in hematology · 2019Review
- Unravel the molecular mechanism of XBP1 in regulating the biology of cancer cells.Journal of Cancer · 2019Review
- Plasmablastic lymphoma: current perspectives.Blood and lymphatic cancer : targets and therapy · 2018Review
- The clinicopathologic spectrum of mature aggressive B cell lymphomas.Virchows Archiv : an international journal of pathology · 2017Review
- Anaplastic lymphoma kinase-positive large B-cell lymphoma: Clinico-pathological study of 17 cases with review of literature.PloS one · 2017Review
- Plasmablastic lymphoma phenotype is determined by genetic alterations in MYC and PRDM1.Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc · 2017Article
- KSHV Latency Locus Cooperates with Myc to Drive Lymphoma in Mice.PLoS pathogens · 2015Article
- STAT3 mutations are present in aggressive B-cell lymphomas including a subset of diffuse large B-cell lymphomas with CD30 expression.Haematologica · 2014Article
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11 authors at 7 institutions in 4 countries.
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Abstract
ALK-positive large B-cell lymphoma is an aggressive lymphoid neoplasm characterized by a monomorphic proliferation of immunoblast-like cells expressing a plasmablastic phenotype and carrying ALK rearrangements. MYC rearrangements are frequent in plasmablastic lymphomas, advanced plasma cell myelomas and a subgroup of diffuse large B-cell lymphomas, but their presence in ALK-positive large B-cell lymphomas is unknown. MYC expression is downregulated by BLIMP1, a master modulator of plasma cell differentiation. BLIMP1 and MYC are upregulated by STAT3, a signal transducer activated by ALK. To determine the role of BLIMP1, MYC and STAT3 in the pathogenesis of ALK-positive large B-cell lymphomas, we investigated MYC rearrangement and the expression of MYC, phosphorylated STAT3, BLIMP1, PAX5 and XBP1 in 12 ALK-positive large B-cell lymphomas. All cases expressed ALK with a granular cytoplasmic pattern. Nine cases had a split signal consistent with an ALK rearrangement. Three additional cases showed a deletion of the 5' or 3' end of the ALK probe consistent with cryptic translocation. PAX5 was virtually negative in all cases tested, whereas BLIMP1 was expressed in all tumors and XBP1 in 11 of 12. Phosphorylated STAT3 was observed in all cases with a strong and diffuse nuclear pattern. MYC rearrangements were not identified in any tumor, but MYC gains and amplification were detected in six cases and one case, respectively. MYC protein was expressed in all tumors independently of MYC gene alterations. These results indicate that ALK-positive large B-cell lymphomas express a complete plasmablastic differentiation program but, contrary to plasmablastic lymphomas, do not have MYC rearrangements. STAT3 is constantly activated and may be an alternative mechanism to promote MYC expression in these tumors. The relevance of the ALK/STAT3 pathway in the pathogenesis of ALK-positive large B-cell lymphomas may offer an attractive target for new therapies.
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