Plasmablastic transformation of low-grade CD5+ B-cell lymphoproliferative disorder with MYC gene rearrangements

Pan, Zenggang; Xie, Qingmei; Repertinger, Susan; Richendollar, Bill G.; Chan, Wing C.; Huang, Qin

doi:10.1016/j.humpath.2013.04.008

Cited by 17 publications

(18 citation statements)

References 28 publications

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“…Among the reported cases, most patients showed an aggressive clinical course with increased prolymphocytes or development of Richter's transformation. 9,[12][13][14]16 In our cohort, 14 patients (70%) showed increased prolymphocytes and/or developed Richter's transformation. Also note that almost half of the patients in our cohort had 14,16).…”

Section: Discussionmentioning

confidence: 99%

“…All cases assessed were negative for CD10. Of note, all these patients (cases 9,11,13,19,20) with an immunophenotype different from chronic lymphocytic leukemia had a typical chronic lymphocytic leukemia immunophenotype at the initial diagnosis.…”

Section: Morphological and Immunophenotypical Findingsmentioning

confidence: 94%

“…MYC immunohistochemistry stain was performed in three patients (cases 13,19,20), and showed a positive expression of MYC in all three cases.…”

Section: Morphological and Immunophenotypical Findingsmentioning

confidence: 99%

“…Prolymphocytes in peripheral blood were increased in nine patients, five with prolymphocytes of 10~34% (chronic lymphocytic leukemia with increased prolymphocytes), and four had prolymphocytes 455% (chronic lymphocytic leukemia in prolymphocytoid transformation) ( Table 1). All patients showed an increased serum B2 microglobulin level (median 4.6 mg/l, range 2.1-11.2 mg/l) (reference range, 0.7-1.8 mg/l) and 17 (cases [5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] had an increased serum lactate dehydrogenase level (median 1220 IU/l range 507-19328 IU/l) (reference range, 313-618 IU/l). Rai stage was IV in nine patients, III in four patients, II in six patients, and 0 in one patient.…”

Section: Clinical and Laboratory Findingsmentioning

confidence: 99%

“…7 8q24/MYC rearrangement is rare in chronic lymphocytic leukemia and only occasional cases have been reported. [8][9][10][11][12][13][14][15][16][17] In most of these reports, 8q24/MYC rearrangement occurred as a part of a complex karyotype, often associated with increased prolymphocytes or Richter's transformation and an aggressive clinical course; only rare cases reported had typical chronic lymphocytic leukemia morphology and responded well to chemotherapy. 5,9 To better characterize the clinical behavior and pathological features of chronic lymphocytic leukemia with 8q24/MYC rearrangement, we searched our database for all chronic lymphocytic leukemia patients at our institution in the past 15 years, and identified 20 patients with 8q24/MYC rearrangement who had detailed clinical, pathological, and cytogenetic information available.…”

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confidence: 99%

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The clinical significance of 8q24/MYC rearrangement in chronic lymphocytic leukemia

Wang

et al. 2016

Modern Pathology

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Chromosome 8q24/MYC rearrangement is associated with Burkitt lymphoma and some aggressive B-cell lymphomas, but is rare in chronic lymphocytic leukemia. We here report a cohort of 20 chronic lymphocytic leukemia patients with 8q24/MYC rearrangement, 3 detected at time of initial diagnosis and 17 acquired after a median interval of 48 months. At the time when 8q24/MYC arrangement was detected, 18 patients had B-symptoms, 17 had lymphadenopathy, and 17 had splenomegaly. Histologically, typical chronic lymphocytic leukemia morphology was seen in six patients, increased prolymphocytes in nine and Richter's transformation in five patients. Eighteen patients had karyotypic information available that showed t(8;v) in a complex karyotype in 12 patients and in a non-complex karyotype in 6 patients. Fluorescence in situ hybridization confirmed MYC rearrangement in 17/17 patients. All patients required therapy after 8q24/MYC rearrangement was detected. At last follow-up, five of six patients with a non-complex karyotype were alive after a median of 74 months (10~143 months) from the detection of 8q24/MYC rearrangement. In contrast, 10 of 12 patients with a complex karyotype died with a median survival of 5.5 months. We conclude that 8q24/MYC rearrangement in chronic lymphocytic leukemia is rare and often acquired during the course of disease. If it is presented in a complex karyotype, it is often associated with Richter's transformation, refractory to therapy and an aggressive clinical course; on the other hand, if it is present in a non-complex karyotype, patients often respond to risk-adapted therapies and achieve remission.

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Section: Discussionmentioning

confidence: 99%

Section: Morphological and Immunophenotypical Findingsmentioning

confidence: 94%

“…MYC immunohistochemistry stain was performed in three patients (cases 13,19,20), and showed a positive expression of MYC in all three cases.…”

Section: Morphological and Immunophenotypical Findingsmentioning

confidence: 99%

Section: Clinical and Laboratory Findingsmentioning

confidence: 99%

mentioning

confidence: 99%

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The clinical significance of 8q24/MYC rearrangement in chronic lymphocytic leukemia

Wang

et al. 2016

Modern Pathology

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Histologic transformation of chronic lymphocytic leukemia/small lymphocytic lymphoma

et al. 2016

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Although generally considered a clinically indolent neoplasm, CLL/SLL may undergo transformation to a clinically aggressive lymphoma. The most common form of transformation, to DLBCL, is also known as Richter syndrome. Transformation determines the course of the disease and is associated with unfavorable patient outcome. Precise detection of transformation and identification of predictive biomarkers and specific molecular pathways implicated in the pathobiology of transformation in CLL/SLL will enable personalized therapeutic approach and provide potential avenues for improving the clinical outcome of patients. In this review, we present an overview of the pathologic features, risk factors, and pathogenic mechanisms of CLL/ SLL transformation.

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Molecular characterization and clonal evolution in Richter transformation: Insights from a case of plasmablastic lymphoma (RT‐PBL) arising from chronic lymphocytic leukaemia (CLL) and review of the literature

Ramsey,

Sabatini,

Smith

et al. 2023

eJHaem

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Plasmablastic transformation of low-grade CD5+ B-cell lymphoproliferative disorder with MYC gene rearrangements

Cited by 17 publications

References 28 publications

The clinical significance of 8q24/MYC rearrangement in chronic lymphocytic leukemia

The clinical significance of 8q24/MYC rearrangement in chronic lymphocytic leukemia

Histologic transformation of chronic lymphocytic leukemia/small lymphocytic lymphoma

Molecular characterization and clonal evolution in Richter transformation: Insights from a case of plasmablastic lymphoma (RT‐PBL) arising from chronic lymphocytic leukaemia (CLL) and review of the literature

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