The Use of R-Hyper-CVAD in a Rare Case of Primary Bone Marrow Diffuse Large B-Cell Lymphoma

Reed, Allison; Sommerhalder, David

doi:10.14740/jh559

Cited by 4 publications

(4 citation statements)

References 5 publications

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“…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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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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“…26,27 Reed et al demonstrated good response to rituximab, cyclophosphamide, vincristine, doxorubicin, and dexamethasone (R-Hyper-CVAD) in a young woman with PBM-DLBCL and high-risk of CNS disease. 28 PBML has been reported to have a median overall survival (OS) ranging from 8 months to 1.8 years. 3,6,7,11,29 According to the IELSG, prognosis differs by histologic subtype with a median OS <1.5 years for those with PBM-DLBCL.…”

Section: Dovepressmentioning

confidence: 99%

Primary Bone Marrow Lymphoma: De Novo and Transformed Subtypes

Kimbrough¹,

Jiang²,

Parent³

et al. 2022

JBM

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Diffuse large B-cell lymphoma (DLBCL) is well known for selectively involving certain extranodal locations such as the central nervous system (CNS), testes, and skin. DLBCL or high-grade B-cell lymphoma selectively involving the bone marrow is rare and has been sparsely reported in the medical literature. We report two cases of lymphoma presenting with primary bone marrow involvement without evidence of involvement of any other sites. The first case represents de novo DLBCL. The patient achieved complete remission with initial treatment, had a bone marrow-only relapse three years later, and achieved a second complete remission following non-transplant salvage therapy. The second case had findings consistent with "double hit" Richter's transformation of chronic lymphocytic leukemia with translocation of c-MYC and BCL-2. This patient had an aggressive clinical course characterized by rapid progression with CNS involvement within three months resulting in the demise of the patient. These two cases represent two distinct subtypes of primary bone marrow lymphoma: de novo and transformed. Further research is necessary to gain a better understanding of this rare lymphoma entity and develop novel therapies. Keywords: diffuse large B-cell lymphoma, high-grade B-cell lymphoma with MYC and BCL-2 rearrangements, bone marrow involvement, cytopenias Plain Language Summary• Primary bone marrow lymphoma (PBML) is confined to the bone marrow with or without a leukemic component and represents a distinct, aggressive type of extranodal lymphoma. • PBML is very rare and has been sparsely reported in the medical literature.• PBML appears to consist of two subtypes: de novo PBML and PBML derived from transformation of bone marrow-based lowgrade lymphomas. • PBML should be distinguished from other aggressive lymphomas which can primarily involve the bone marrow such as Burkitt lymphoma, lymphoblastic lymphoma, blastoid mantle cell lymphoma, and intravascular large B-cell lymphoma. • PET imaging is useful for monitoring lymphoma activity in the bone marrow.

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“…PBML is considered as a distinct subtype of DLBCL with poor prognosis 1,2 . The use of intensive induction therapies such as R‐Hyper CVAD or R‐CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone) followed by up‐front high‐dose chemotherapy and autologous stem cell rescue has been reported based on the assumption of poor prognosis 3,4 . However, because of its rarity, the clinical features of PBML remain unclear and there is no evidence base to support a recommendation of intensive induction therapy.…”

Section: Introductionmentioning

confidence: 99%

Early complete response of primary bone marrow B‐cell lymphoma treated with rituximab‐based CHOP therapy, assessed by flow cytometry and immunogloblin heavy chain rearrangement

Nabe

Kikuchi

Kamihara

et al. 2021

Clinical Case Reports

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The Use of R-Hyper-CVAD in a Rare Case of Primary Bone Marrow Diffuse Large B-Cell Lymphoma

Cited by 4 publications

References 5 publications

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

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

Primary Bone Marrow Lymphoma: De Novo and Transformed Subtypes

Early complete response of primary bone marrow B‐cell lymphoma treated with rituximab‐based CHOP therapy, assessed by flow cytometry and immunogloblin heavy chain rearrangement

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