Leukemic phase of CD5+ diffuse large B cell lymphoma

Hsiao, Hui‐Hua; Wang, Hui‐Ching; Tsai, Yu-Fen; Hsiao, Chien; Cho, Shih‐Feng; Liu, Yi-Chang

doi:10.4274/tjh.2016.0447

Cited by 3 publications

(3 citation statements)

References 3 publications

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“…Therefore, a leukemic phase of a CD5+ positive DLBCL seems to occur very rarely. 10 , 12 , 13 Yamaguchi et al 10 reported 4 cases of CD5+ DLBCL in leukemic phase in a series of 120 patients: thus, a prevalence of about 3.3% might be calculated.…”

Section: Discussionmentioning

confidence: 99%

A rare case of de novo CD5+ diffuse large B-cell lymphoma in leukemic phase and positive for CD13

et al. 2018

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We report a case of de novo diffuse large B-cell lymphoma (DLBCL) in leukemic phase, positive for both CD5 and CD13. Morphologic evaluation, flow cytometric immunophenotyping, karyotyping and polymerase chain reaction studies were performed. Neoplastic lymphocytes appeared as blast-like cells, positive for CD19, CD20, CD5, CD13, CD79a, HLADR, and with restriction for surface immunoglobulin K light chains. Rearrangement of IgH gene, BCL2/IgH translocation and complex karyotype were found. The patient was treated with RCOMP regimen and achieved complete remission. However, only one month after the first restaging of disease, the patient presented with symptoms attributable to central nervous system involvement and her clinical conditions worsened rapidly. While both CD5 expression and leukemic presentation are uncommon findings in DLBCL, positivity for CD13 is very rare. The outcome of our patient shows the poor prognosis of CD5+ DLBCL with leukemic presentation. The possible role of CD13 coexpression is discussed.

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Section: Discussionmentioning

confidence: 99%

A rare case of de novo CD5+ diffuse large B-cell lymphoma in leukemic phase and positive for CD13

et al. 2018

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“…Using a Medline search with the key words “diffuse large B-cell lymphoma AND leukemic/leukemic phase” and “diffuse large B-cell lymphoma AND leukemic/leukemic presentation”, we identified a total of 16 case reports and 2 case series of de novo or relapsed DLBCL presenting in the leukemic phase that were published in English ( Table 1 ). 7 - 24 In one case series describing the clinical course of 40 individuals with leukemic presentations of de novo DLBCL, 32 (80%) patients died with a median follow-up of 18 months. 23 The median follow-up for the 8 surviving patients was 34.5 months.…”

Section: Discussionmentioning

confidence: 99%

A Clinical Triad with Fatal Implications: Recrudescent Diffuse Large B-cell Non-Hodgkin Lymphoma Presenting in the Leukemic Phase with an Elevated Serum Lactic Acid Level and Dysregulation of the TP53 Tumor Suppressor Gene – A Case Report and Literature Review

Hwang

Aboulafia

2021

Clin Med�Insights�Blood�Disord

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Despite representing 30% to 40% of newly diagnosed cases of adult non-Hodgkin lymphoma, diffuse large B-cell lymphoma (DLBCL) rarely presents (1) in the leukemic phase (2) with dysregulation of the TP53 tumor suppressor gene and (3) an elevated serum lactic acid level. In this case report and literature review, we highlight this unfortunate triad of poor prognostic features associated with an aggressive and fatal clinical course in a 53-year-old man with recrudescent DLBCL. A leukemic presentation of de novo or relapsed DLBCL is rare and may be related to differential expressions of adhesion molecules on cell surfaces. In addition, TP53 gene mutations are present in approximately 20% to 25% of DLBCL cases and foreshadow worse clinical outcomes. Finally, an elevated serum lactic acid level in DLBCL that is not clearly associated with sepsis syndrome is a poor prognostic factor for survival and manifests as type B lactic acidosis through the Warburg effect.

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“…The literature is also unclear as to the optimal treatment regimens for the combined presentation of CD5+ DLBCL with leukemic transformation. An increasing number of case reports separately detail both DLBCL with leukemic transformation [7][8][9][10][11] and CD5 positivity [4,12], but mentions of dual presentation have been limited, as have case series and larger studies [5][6]. The authors of these reports, where treatment is discussed, ultimately favor an anthracycline-based treatment regimen, often paired with rituximab, though R-hyper-CVAD with alternating IT MTX/cytarabine [13] has also been reported.…”

Section: Discussionmentioning

confidence: 99%

CD5+ Diffuse Large B-Cell Lymphoma With Leukemic Transformation: A Rare Case With Central Nervous System Involvement, Treated With R-CHOP and Intrathecal Methotrexate/Cytarabine

Cordova

Nelson

Brizendine

et al. 2021

Cureus

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Diffuse large B-cell lymphoma (DLBCL) is one of several subtypes of non-Hodgkin's lymphoma, and one that can present in a myriad of ways. One unique and particularly aggressive presentation is leukemic transformation with CD5 positivity, which leads to systemic symptoms, a relatively high peripheral tumor load, and higher rates of CNS involvement. The prevalence of leukemic transformation has not been determined, as published literature is limited to case reports and small case series. CD5 positivity appears to be even rarer and is only found in a small fraction of DLBCL with leukemic transformation. Treatment regimens for this presentation have not been well-established due to the rarity of the disease and paucity of literature on the subject. Our patient, a 76-year-old female with a history of previously treated stage IIIB follicular lymphoma, was found to have CD5+ DLBCL with leukemic transformation. She was treated with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) along with intrathecal methotrexate (IT MTX)/cytarabine after CNS involvement was diagnosed. The patient tolerated therapy well, with an objective reduction in leukocytosis and blast count. To our knowledge, this is the first such case of CD5+ DLBCL with leukemic transformation treated with dose-reduced R-CHOP and IT MTX/cytarabine. Her response to therapy indicates that this regimen could be a viable option for the treatment of this exceedingly rare disease presentation.

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Leukemic phase of CD5+ diffuse large B cell lymphoma

Cited by 3 publications

References 3 publications

A rare case of de novo CD5+ diffuse large B-cell lymphoma in leukemic phase and positive for CD13

A rare case of de novo CD5+ diffuse large B-cell lymphoma in leukemic phase and positive for CD13

A Clinical Triad with Fatal Implications: Recrudescent Diffuse Large B-cell Non-Hodgkin Lymphoma Presenting in the Leukemic Phase with an Elevated Serum Lactic Acid Level and Dysregulation of the TP53 Tumor Suppressor Gene – A Case Report and Literature Review

CD5+ Diffuse Large B-Cell Lymphoma With Leukemic Transformation: A Rare Case With Central Nervous System Involvement, Treated With R-CHOP and Intrathecal Methotrexate/Cytarabine

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