scholarly journals A rare case of primary cutaneous diffuse large B cell lymphoma, leg type with metastasis

Author(s):  
Agni K. Bose ◽  
Pandharinath K. Khade ◽  
Vidya D. Kharkar

<p class="abstract">Primary cutaneous diffuse large B cell lymphoma, leg type (PCDLBCL-LT) is a rare and aggressive type of primary cutaneous B cell lymphoma (PCBCL), which represents 10-20% cases of PCBCL. It has a 40-50% recurrence rate and 5 year survival rate of 50%. Here, we present a case of an 86 year old female who presented to us with complaints of slightly tender annular plaques with an oedematous base present over bilateral lower limbs and pitting oedema. Histopathological examination from the annular lesion showed normal epidermis, grenz zone and a dense lymphoid infiltrate involving almost the entire dermis. Immunohistochemistry confirmed histological findings, atypical cell were positive for CD20 and MUM1 protein with focal expression of BCL 6 which is rare. Based on the above findings, we made a diagnosis of diffuse large B cell lymphoma-leg type and started her on palliative radiotherapy. As PCBCL-LT is rare and aggressive lymphoma, we present this case to review literature and summarise its clinical features.</p>

2021 ◽  
Vol 15 ◽  
pp. 117822342110507
Author(s):  
Nitya Prabhakaran ◽  
Hassan Sheikh ◽  
Xinmin Zhang ◽  
Silvat Sheikh-Fayyaz

Intravascular large B-cell lymphoma (IVLBCL) is a rare and high-grade disease of neoplastic lymphoid cells within the vascular lumina of small- to medium-sized vessels. The disease carries a grim prognosis despite robust treatment protocols. We discuss the case of a 58-year-old female who presented with mammographic screening abnormality which led to more investigations and ultimately to this diagnosis. The patient had no prior history of a lymphoma or in situ and invasive carcinoma of the breast. To our knowledge, IVLBCL of the breast is a very rare and an unusual location for this type of a lymphoma and so far, only five reported cases. Through our case report, we not only discuss the case but also review literature on this rare entity.


2021 ◽  
Vol 14 (3) ◽  
pp. e239422
Author(s):  
Midori Tokushima ◽  
Masaki Tago ◽  
Naoko E Katsuki ◽  
Shu-ichi Yamashita

A 75-year-old woman developed redness and swelling on her truncal skin, spreading from the lower abdomen to left thigh, 2 months before being admitted to our hospital. She was urgently hospitalised because of her worsening respiratory condition. On admission, she had reticular telangiectasia, diffuse skin induration on the lower abdomen and panhypopituitarism. She was diagnosed with intravascular large B-cell lymphoma (IVLBCL) by the third random abdominal skin biopsy. After histopathological examination at autopsy, we made a final diagnosis of IVLBCL causing respiratory failure and panhypopituitarism. This is the rare case of IVLBCL-induced panhypopituitarism showing visible skin lesions.


2020 ◽  
pp. OP.20.00362
Author(s):  
Urshila Durani ◽  
Dennis Asante ◽  
Herbert C. Heien ◽  
Carrie A. Thompson ◽  
Thorvardur Halfdanarson ◽  
...  

In 2013, the American Society of Hematology (ASH) published recommendations with Choosing Wisely to limit surveillance imaging in aggressive lymphoma. We studied surveillance imaging practice patterns for diffuse large B-cell lymphoma (DLBCL) before and after the ASH Choosing Wisely campaign. We used OptumLabs Data Warehouse, a national insurance claims database, to retrospectively study imaging frequency in survivors of DLBCL from 2008 to 2016. Three time periods were defined: Period 1 (2008 to 2010), Period 2 (2011 to 2013), and Period 3 (2014 to 2016). One thousand four hundred seventy-two patients were included. Median follow up was approximately 2 years. During the first and second years of surveillance, imaging remained stable between Period 1 (years 1 and 2: 199 [91%] and 137 [83%], respectively) and Period 2 (years 1 and 2: 257 [88%] and 172 [77%], respectively; P = .38), but decreased in Period 3 (years 1 and 2: 315 [78%] and 83 [61%], respectively; P < .01). In a multivariable logistic regression, year after 2012 was a significant predictor of decreased overuse (more than two scans per year in the first year of surveillance; [odds ratio, 0.49 for 2013 v 2008; P = .02]). Our study demonstrated the rate of surveillance scans—both computed tomography and positron emission tomography imaging—in DLBCL decreased after the ASH Choosing Wisely campaign. Multiple factors, such as changes in recommendations, reimbursement, and provider knowledge base, may have all contributed and should be studied further.


Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 4931-4931
Author(s):  
Yoshiki Terada ◽  
Hirohisa Nakamae ◽  
Ran Moriguchi ◽  
Hiroshi Kanashima ◽  
Erina Sakamoto ◽  
...  

Abstract Background. Recently several retrospective studies showed that relative dose intensity (RDI) in combination chemotherapy including CHOP significantly influences survival in aggressive lymphoma. Based on these data, maintaining high RDI in chemotherapy by, for example, prophylactic granulocyte colony-stimulating factor (G-CSF) administration has been attempted to obtain better outcome. Moreover, rituximab, a chimeric monoclonal anti CD20 antibody combined with CHOP chemotherapy (R-CHOP) has significantly ameliorated the outcome in patients with diffuse large B-cell lymphoma (DLBL). However, it is unclear if higher RDI even in combination with rituximab will improve outcome in B cell type aggressive lymphoma. Hence, in the current study, we retrospectively analyzed the impact of RDI in R-CHOP as an initial treatment on survival of patients with DLBL. Furthermore, we determined the factors influencing RDI. Patients and Methods. We studied 100 previously untreated DLBL patients who underwent more than 3 courses of R-CHOP chemotherapies at 5 institutions from December 2003 to February 2008. The median age of the patients was 60 years old (range 19–79). The median number of R-CHOP course was 6 (range, 3–8). In the current study, the RDI was calculated by averaging the delivered RDIs of cyclophosphamide (CY) and adriamycin (ADR) for all chemotherapy courses. Results. The median average RDI of CY and ADR (CY/ADR-RDI) in all patients was 87.9%. Twenty three of 100 patients were treated with RDI less than 75 %. With a median follow-up of 21.2 months, the probability of 4-year overall survival (OS) was significantly higher in patients with higher RDI (&gt;=75%) than that in patients with lower RDI (&lt;75%) (78.6 % vs. 60%; P = 0.01). In univariate Cox regression model to identify prognostic factors for OS, RDI [hazard ratio (HR) = 0.7 per 0.1 of RDI; 95% CI 0.6– 0.9; P = 0.02] and high/high-intermediate International Prognostic Index (IPI) (HR = 4.7; 95% CI 1.3–17; P = 0.04) were significant factors influencing OS. In multivariate model, RDI was only a significant factor influencing OS (HR = 0.8 per 0.1 of RDI; 95% CI 0.6–1.0; P = 0.04). In multivariate logistic analysis to determine factors influencing RDI, elderly patients (&gt;=51 ) [odds ratio (OR) = 0.2; 95% CI 0.1–0.7; P = 0.01] and high/high-intermediate IPI (OR = 0.3; 95% CI 0.1–1.0; P = 0.04) were significant factors for reduced RDI, whereas prophylactic G-CSF (OR = 3.2; 95% CI 1.1–9.3; P = 0.04) was found to be a significant factor for increased RDI. Conclusion. In newly diagnosed DLBL patients, the current results demonstrated that high RDI in CHOP even when combined with rituximab was significantly associated with better survival and higher RDI could be effectively maintained by G-CSF.


2018 ◽  
Vol 12 (3) ◽  
pp. 699-703 ◽  
Author(s):  
Toshiro Sugiyama ◽  
Kotaro Arita ◽  
Eiji Shinno ◽  
Takahiko Nakajima

According to the literature, spontaneous remission of aggressive lymphomas is extremely rare; gastric non-Hodgkin lymphomas, such as mucosa-associated lymphoid tissue lymphomas, often regress due to Helicobacter pylori treatment or no progression, even in a watch-and-wait strategy. Although spontaneous remission of diffuse large B cell lymphomas in the stomach was very rarely reported, the follow-up periods of the cases of spontaneous remission are within 2 years and most cases are likely to relapse after the first remission. Here, we report that a diffuse large B cell lymphoma in the stomach showed spontaneous remission within 2 months after the initial diagnosis and the remission is still continuing for 10 years without any specific treatments against this aggressive lymphoma.


Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 3660-3660 ◽  
Author(s):  
Nirmeen Ali Megahed ◽  
Seiichi Kato ◽  
Naoko Asano ◽  
Shigeo Nakamura

Abstract Abstract 3660 Background: Recently more light has been shed on the overlap in biologic and morphologic features between classic Hodgkin lymphoma (cHL) and B-cell non-Hodgkin lymphoma. This overlap was further substantiated by analyzing the neoplastic nature of the malignant cell of cHL, which was proven to be of B-cell origin in nearly all cases. In addition, the growing recognition of composite and metachronous Hodgkin and non-Hodgkin lymphomas provided a further evidence of such claim. This advocated the term Grey zone lymphoma, which was first introduced in 1998 in the ÔÔWorkshop on HodgkinÕs disease and related diseasesÕÕ. According to WHO classification 2008, the term Ògrey zone lymphomaÓ designates anterior mediatsinal involvement with lymphoma intermediate between DLBCL and cHL. This type of lymphoma is claimed to have a more aggressive course than either cHL or DLBCL. Although the optimum treatment regimen in such cases is not yet well established most authors go for administration of therapy appropriate for DLBCL with the addition of Rituximab. We present a reappraisal of anaplastic variant of diffuse large B-cell lymphoma as an extramediastinal grey zone lymphoma showing morphologic and phenotypic features intermediate between DLBCL and cHL. Methods and results: Clinical and pathological data of 13 cases were retrieved over 8-year period. The age of the patients ranged from 57 to 86 years old (mean= 75 yeas). Eight of our cases were males and 5 were females with slight male predominance (male: female ratio= 1.6:1). This runs in contradiction with cases of mediastinal grey zone lymphoma which show striking female predominance. All the cases were presented with extramediastinal lymphadenopathy. Six patients showed associated extranodal involvement (e.g. liver, stomach, forearm, bone). None of the cases was associated with EBV activity. Four cases were presented with stage IV disease, 3 cases with stage III and 4 cases with stage II. B symptoms were reported in 6 cases. Histopathologically, tumors showed sheet like growth of pleomorphic cells, some of them are Reed Sternberg like cells, admixed with inflammatory background formed of mature lymphocytes eosinophils and plasma cells. Fibrous strands and geographic necrosis were seen in all cases. B-cell immunophenotyping of the tumor cells was evidenced by positivity for CD20, CD79a and Pax 5 in all cases. On the other hand, Hodgkin-like immunophenotyping was evidenced by positivity for CD30 in 10 cases, positivity for CD15 in 2 cases, positivity for Mum1 in one case and positivity for fascin in 6 cases. All the cases were negative for ALK1, CD10, CD3, κ□Aλ and CD45 RO. Worth to be noted that 69% of the cases (9/13) showed positivity for p53 and 23% (3/13) showed positivity for BCL-6 which are both an unusual finding in cHL. Forty six percent of the cases (6/13) died within the first year of the disease course (mean=8.3 months). Six patients are still alive (46%), 5 of which showed complete remission and one patient showed partial remission. Conclusion: Anaplastic variant of diffuse large cell lymphoma shows phenotypic and immunotypic features intermediate between cHL and DLBCL. We here propose the term (Extramediastinal grey zone lymphoma) for such cases which represent a distinctive subgroup of aggressive lymphoma. Further future studies of this group could contribute more to unmasking the link between Hodgkin and Non-Hodgkin lymphomas. Disclosures: No relevant conflicts of interest to declare.


2018 ◽  
Vol 2018 ◽  
pp. 1-4
Author(s):  
Liziane Cattelan Donaduzzi ◽  
Angélica Reinheimer ◽  
Maria Augusta Ramires da Silva ◽  
Lucia de Noronha ◽  
Aline Cristina Batista Rodrigues Johann ◽  
...  

Objective. To report a case of a challenging oral diagnosis involving a primary diffuse large B cell lymphoma of the oral cavity mimicking a hyperplastic reactive lesion.Case Report. A 72-year-old male patient was referred to the Department of Stomatology presenting a proliferative nodular lesion in the anterior region of the mandible involving the anterior teeth. The clinical examination revealed anterior teeth affected by periodontal disease, suggesting the nodular cession hyperplastic reaction. Incisional biopsy was performed under local anesthesia. The histopathological examination revealed a diffuse proliferation of atypical large lymphoid cells. The tumor cells showed immunopositivity for CD20 and Ki67 (100%) and negativity for CD3, CD30, and CD15. The diagnosis of diffuse large B cell lymphoma was established. The patient underwent chemotherapy and progressed to death after nine months.Conclusion. Lymphomas of the oral cavity are rare and may have nonspecific clinical features, mimicking inflammatory and reactive lesions. Therefore, a detailed clinical evaluation associated with histopathological and immunohistochemical analysis should be performed to enable early and accurate diagnoses in suspected oral lesions.


2018 ◽  
Vol 115 (3) ◽  
pp. E498-E505 ◽  
Author(s):  
Li Lu ◽  
Fen Zhu ◽  
Meili Zhang ◽  
Yangguang Li ◽  
Amanda C. Drennan ◽  
...  

STAT3 is constitutively activated in many cancers and regulates gene expression to promote cancer cell survival, proliferation, invasion, and migration. In diffuse large B cell lymphoma (DLBCL), activation of STAT3 and its kinase JAK1 is caused by autocrine production of IL-6 and IL-10 in the activated B cell–like subtype (ABC). However, the gene regulatory mechanisms underlying the pathogenesis of this aggressive lymphoma by STAT3 are not well characterized. Here we performed genome-wide analysis and identified 2,251 STAT3 direct target genes, which involve B cell activation, survival, proliferation, differentiation, and migration. Whole-transcriptome profiling revealed that STAT3 acts as both a transcriptional activator and a suppressor, with a comparable number of up- and down-regulated genes. STAT3 regulates multiple oncogenic signaling pathways, including NF-κB, a cell-cycle checkpoint, PI3K/AKT/mTORC1, and STAT3 itself. In addition, STAT3 negatively regulates the lethal type I IFN signaling pathway by inhibiting expression of IRF7, IRF9, STAT1, and STAT2. Inhibition of STAT3 activity by ruxolitinib synergizes with the type I IFN inducer lenalidomide in growth inhibition of ABC DLBCL cells in vitro and in a xenograft mouse model. Therefore, this study provides a mechanistic rationale for clinical trials to evaluate ruxolitinib or a specific JAK1 inhibitor combined with lenalidomide in ABC DLBCL.


2011 ◽  
Vol 29 (14) ◽  
pp. 1835-1843 ◽  
Author(s):  
Itziar Salaverria ◽  
Reiner Siebert

It has long been recognized that the border between classical Burkitt's lymphoma (BL) and classical diffuse large B-cell lymphoma (DLBCL) is hard to determine. Instead, both classical lymphoma entities seem to be the extreme ends of a spectrum of diseases that contains a group of lymphomas characterized predominately by the fact that they are hard to assign to the one or the other group. This gray zone has been recently termed “lymphoma, unclassifiable, with features intermediate between DLBCL and BL” by the updated WHO classification. The term “intermediate” resembles that from a recent gene-expression study of mature aggressive B-cell lymphomas, although, notably, it is used differently. Intermediate lymphomas according to the WHO classification clearly are a temporary container of different biologic subtypes of aggressive lymphoma, from which several might be associated with an unfavorable clinical outcome. The present review aims at describing the morphologic, clinical, and biologic heterogeneity of the intermediate lymphomas and, moreover, attempts to propose testable subgroups based on age and presence of genetic aberrations.


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