&lt;p&gt;Clinical features and prognostic factors of primary bone marrow lymphoma&lt;/p&gt;

Wang, Gangjian; Chang, Yu; Wu, Xiaolong; Li, Xin; Li, Ling; Zhang, Lei; Fu, Xiaorui; Sun, Zhenchang; Zhang, Xudong; Zhang, Mingzhi

doi:10.2147/cmar.s187522

Cited by 10 publications

(26 citation statements)

References 50 publications

(30 reference statements)

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“…In particular, race was identified as a prognostic factor and this may be related to genotypic milieu and gene specificity. Lymphoma at different primary sites has different clinical features and prognosis (32,33), even though primary sites is not significant in the Cox multivariate analysis, it was still imported into the nomogram prediction. The nomograms of OS and CSS were constructed and were used to predict prognosis with exact scores corresponding to different survival rates, which was validated both internally and externally via C-indexes and calibration plots.…”

Section: Discussionmentioning

confidence: 99%

Status and prognostic nomogram of patients with Burkitt lymphoma

et al. 2019

Oncol Lett

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The purpose of the present study was to evaluate the newest status of patients diagnosed Burkitt lymphoma (BL), an aggressive lymphoma subset with a high cure rate. Furthermore, the study aimed to create prognostic nomograms to consider various prognostic factors and estimate patient survival, paving the way for clinical decision-making. A total of 4,600 patients diagnosed with BL between 1983 and 2015 were investigated, via data collected from the SEER database. The overall status of the patients was analyzed through several aspects, including incidence and survival analysis of the previous three decades using the log-rank test and the Kaplan-Meier method. In order to construct and validate the nomograms, the patient diagnosed during 2005–2015 were randomly assigned to the training cohort and validation cohort. Univariate and multivariate analyses were applied to identify independent factors that were further included in the nomograms, predicting 3- and 5-year overall survival (OS) and cancer-specific survival (CSS). The data of the training cohort were used for internal validation and validation cohort used to external validation. C-index and calibration plots were used to validate the nomograms, comparing predicted values with actual outcomes. The incidence of BL was gradually increased from 1984 and reached its peak in 2009, at a rate of 0.491 per 100,000 [95% confidence interval (CI), 0.412–0.581]. From 2009, the incidence slowly declined year by year and dropped to 0.280 per 100,000 (95% CI, 0.224–0.346). The OS and CSS rates of patients diagnosed between 2005 and 2015 were increased, in contrast with those of patients diagnosed from 1983–1993 and 1994–2004. A total of five variables, including age, race, chemotherapy, primary site and stage, proved to be the prognostic factors of BL and were used to construct the nomograms predicting 3- and 5-year OS and CSS. The internal and external calibration plots for the probability of 3- and 5-year OS and CSS were consistent between nomogram prediction and observed outcomes. The slow decline in incidence and the significantly improved cure rate make BL a disease that is no longer an urgent problem. Effective nomograms were developed to predict the OS and CSS of patients with BL.

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Section: Discussionmentioning

confidence: 99%

Status and prognostic nomogram of patients with Burkitt lymphoma

et al. 2019

Oncol Lett

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“…There are also other types, including Hodgkin’s lymphoma, peripheral T-cell lymphoma, ALK-negative anaplastic large cell lymphoma, and FL. 3…”

Section: Discussionmentioning

confidence: 99%

“…There were 51 high-grade BCL cases, and among these 77% (48/62) were DLBCL and 5% (3/62) were left unclassifiable. 1,3,5-33 Meanwhile, there were 11 cases of low-grade BCL, and 11% (7/62) were FLs. 1,34,35 According to the character of our case, we focused more on the analysis of HGBL.…”

Section: Discussionmentioning

confidence: 99%

Aggressive Diffuse Intermediate Size B-Cell Lymphoma With P53 Mutation Presented as Primary Bone Marrow Lymphoma

Chen

Jorsan

Avezbakiyev

et al. 2020

Journal of Investigative Medicine High Impact Case Reports

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Primary bone marrow lymphoma (PBML) is a disease entity in which lymphoma primarily originates in the bone marrow without signs of involvement of lymph nodes, spleen, liver, or any other organs, and excludes leukemia/lymphoma. PBML has been a rare presentation of malignant lymphoma, and most of the cases have a poor prognosis and require rapid diagnoses and treatments. Among all PBMLs, diffuse large B-cell lymphoma (DLBCL) is the most common pathological subtype. Over 25 years and from 7 institutions, the International Extranodal Lymphoma Study Group retrospectively collected PBML cases and, in 2012, published these 21 cases, including 19 cases of B-cell lymphoma and 2 cases of peripheral T-cell lymphoma. Among the B-cell types, DLBCL accounted for 79% and follicular lymphoma (FL) for 21%. DLBCLs were characterized by the existence of large cells. In this article, we present a rare case of high-grade aggressive type with P53 mutation, intermediate-sized B-cell lymphoma, excluded FL by the absence of FL lymphoma markers, presented as PBML. Our patient had rapid progression and succumbed to the disease shortly after diagnosis. Upon literature review, 62 B-cell lymphoma cases were identified that presented as PBML (51 high-grade and 11 low-grade)—mostly case reports. Among these, only one case was reported as intermediate-sized DLBCL-like lymphoma but not with aggressive features. Our case represents the first case of aggressive intermediate-sized lymphoma, not a FL, with P53 mutation, highly elevated lactate dehydrogenase, and Ki-67 presented as PBML. Such a profile would need to be quickly recognized and aggressive treatment applied, such as CART (chimeric antigen receptor T-cells) therapy or DA-EPOCH-R (dose-adjusted EPOCH [etoposide-prednisone-oncovin-cyclophosphamide-hydroxydaunorubicin] and rituximab) with or without venetoclax.

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“…The 2017 WHO classification of haematological neoplasms provides no official definition of PBM-DLBCL, which would currently be classified as DLBCL NOS according to current WHO definitions. Several published definitions consist of a spectrum comprising 'purist' approaches, such as the one adopted by the International Lymphoma Study Group, 16,17 not allowing any localisation beyond the BM, and more 'relaxed' definitions, for instance allowing for hepatosplenomegaly 18 or sometimes even for local lymphadenopathy. 19 More complexity is added by the distinction between PBM-DLBCL and PB-DLBCL, whereby the latter (recognised as an entity in the WHO classification of tumours of soft tissues and bone 20 ) presents with single or multiple lytic lesions (involving, de facto, also the BM) and the former presents with disseminated BM disease in the absence of osteolysis, even at the microscopic level.…”

Section: Pbm-dlbcl Iv-lbcl and Large B-cell Lymphoma Presenting In mentioning

confidence: 99%

“…The reported patterns of BM involvement by PBM-DLBCL are nodular and/or diffuse or, rarely, intrasinusoidal. 16,21 From the best characterised series, it seems that almost equal proportions of GCB and non-GCB cases are present, 16 although a recent analysis of published cases reported CD10 expression in an overall minimal percentage of cases, whereas most of them expressed IRF4/MUM1, 17 with most cases therefore being classified as non-GCB according to the Hans algorithm. 22 IV-LBCL is defined, according to the WHO, as an extranodal lymphoma characterised by selective growth within the lumina of vessels, with the exception of larger arteries and veins.…”

Section: Pbm-dlbcl Iv-lbcl and Large B-cell Lymphoma Presenting In mentioning

confidence: 99%

Aggressive B‐cell lymphomas with a primary bone marrow presentation

et al. 2020

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Aggressive B‐cell lymphomas present as a heterogeneous spectrum of disease. A primary diagnosis in the bone marrow (BM) may be challenging in terms of diagnostic classification and clinical handling, owing to limited architectural information. Aggressive B‐cell lymphomas can be subdivided into entities that typically present primarily in the BM, and cases with BM involvement in which the bulk of disease is present in other organs. One main topic at the 2018 BM workshop of the European Association of Haematopathology/Society of Hematopathology was therefore aggressive B‐cell lymphomas with a primary BM presentation. The spectrum of cases submitted to this topic gave a good overview of commonly encountered problems, as well as unusual manifestations, and highlighted areas of imprecise disease definitions and diagnostic grey zones. The categories submitted to the workshop included cases of Burkitt lymphoma (BL) with unusual features, high‐grade B‐cell lymphomas (HG‐BCLs) with and without so‐called double/triple‐hit, and diffuse large B‐cell lymphomas (DLBCLs) with a primary BM presentation. Areas of difficulties included the morphological boundaries of HG‐BCL not otherwise specified, cases with MYC and bcl‐2 or bcl‐6 translocations and terminal deoxynucleotidyl transferase (TdT) expression, which were categorised as B‐cell lymphoblastic leukaemia/lymphoma if most cells showed TdT positivity, and the clinicopathological overlap between intravascular large B‐cell lymphoma, CD5‐positive DLBCL, and DLBCL with primary presentations in the BM, spleen, and liver. This review summarises our understanding of the main aggressive B‐cell lymphoma categories with a common primary BM presentation and potential problem areas, and makes suggestions for the immunophenotypic and genetic work‐up, illustrated by the interesting and challenging cases submitted to the workshop.

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<p>Clinical features and prognostic factors of primary bone marrow lymphoma</p>

Cited by 10 publications

References 50 publications

Status and prognostic nomogram of patients with Burkitt lymphoma

Status and prognostic nomogram of patients with Burkitt lymphoma

Aggressive Diffuse Intermediate Size B-Cell Lymphoma With P53 Mutation Presented as Primary Bone Marrow Lymphoma

Aggressive B‐cell lymphomas with a primary bone marrow presentation

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