Diffuse large B-cell lymphoma in blood and bone marrow: a rare presentation of leukemic phase at diagnosis

Suresh, Pooja K; Basavaiah, Sridevi Hanaganahalli; Goel, Mrigank; Dsouza, Sanyo; Rai, Santosh

doi:10.1016/j.htct.2019.04.005

Cited by 4 publications

(3 citation statements)

References 9 publications

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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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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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“…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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“…Its prognosis is based on the International Prognostic Index (IPI) score and genomic markers, and it is treated with anthracycline/rituximab-based chemotherapy. However DLBCL presenting in leukemic phase is extremely rare and can be challenging to differentiate from acute leukemia or other types of NHL [ 2 - 4 ]. Therefore, to obtain an accurate diagnosis immunophenotyping and cytogenetic analyses must be performed.…”

Section: Introductionmentioning

confidence: 99%

MYC-Positive Diffuse Large B-Cell Lymphoma in Leukemic Phase at Presentation: A Diagnostic and Therapeutic Challenge

Bilgin

Castel

2021

J Hematol

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Diffuse large B-cell lymphoma in blood and bone marrow: a rare presentation of leukemic phase at diagnosis

Cited by 4 publications

References 9 publications

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

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

MYC-Positive Diffuse Large B-Cell Lymphoma in Leukemic Phase at Presentation: A Diagnostic and Therapeutic Challenge

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