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

(4 citation statements)

References 9 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…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

View full text Add to dashboard Cite

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.

show abstract

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

View full text Add to dashboard Cite

show abstract

“…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

View full text Add to dashboard Cite

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.

show abstract

“…Diffuse large B-cell lymphoma (DLBCL) comprises 25%-35% of adult non-Hodgkin lymphomas and is a highly heterogenous group of lymphoid malignant disorders. Although the involvement of bone marrow with DLBCL cells circulating in peripheral blood is not uncommon in progressive or stage IV disease, initial presentation in the leukemic phase is rare, mostly having been documented in case reports or small study populations (Muringampurath-John et al 2012;Suresh et al 2020). Previous attempts to define distinctive features of leukemic DLBCL by flow cytometry have been inconclusive (Muringampurath-John et al 2012).…”

Section: Introductionmentioning

confidence: 99%

Leukemic presentation and progressive genomic alterations of MCD/C5 diffuse large B-cell lymphoma (DLBCL)

Kim,

Nejati,

et al. 2023

Cold Spring Harb Mol Case Stud

View full text Add to dashboard Cite

Diffuse large B-cell lymphoma (DLBCL) is a very heterogenous group, subdivided into germinal-center (GC)-derived and activated B-cell (ABC) types. Advances in molecular methodologies, including whole exome sequencing (WES) and chromosomal microarrays (CMA), have fostered molecular subclassification of DLBCL, while improving our understanding of their pathogenic mechanisms and resistance to therapy. Here we present distinct case of de novo DLBCL that presented in leukemic form. WES revealed point mutations of CD79B, MyD88, TP53, TBL1XR1 and PIM1 genes, indicating that this lymphoma with leukemic presentation fits the best the MCD/C5 molecular subtype of DLBCL, the prominent subcategory of the ABC DLBCL. High-resolution CMA revealed amplification of genomic regions containing BTK, CCDN3, and PIM1 genes and loss of CDNK2A gene. Despite an initial good clinical response to BTK inhibitor ibrutinib, anti-CD20 antibody rituxan, alkylating agent bendamustine, and hematopoietic stem-cell transplant, the lymphoma relapsed, accompanied by morphologic and molecular evidence of disease progression. BTK and FOXO1 gene mutations emerged, indicative of ibrutinib and rituxan resistance, respectively, with CMA indicating also partial loss of BTK gene amplification. The recurrent tumor developed loss of TP53 heterozygosity and additional chromosomal changes, considered central to ABC DLBCL pathogenesis, such as PRDM1 loss. Finally, the relapsed lymphoma cells showed in vitro resistance to standard BTK inhibitors but sensitivity to vecabrutinib, active against mutated BTK, and to PIM1 inhibitor. In summary, we provide in-depth molecular characterization of a case representing leukemic form of DLBCL and discuss mechanisms that may have contributed to lymphoma progression and development of drug resistance.

show abstract

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

Leukemic presentation and progressive genomic alterations of MCD/C5 diffuse large B-cell lymphoma (DLBCL)

Contact Info

Product

Resources

About