RESUMO
DISEASE OVERVIEW: Plasmablastic lymphoma (PBL) is a rare CD20-negative aggressive lymphoma with a poor prognosis under standard treatment options. Though PBL is associated with human immunodeficiency virus infection and other immunosuppressed states, it can also affect immunocompetent individuals. DIAGNOSIS: The diagnosis requires a high clinical suspicion and pathological confirmation. EBER expression and MYC gene rearrangements are frequently detected. The differential diagnosis includes EBV+ diffuse large B-cell lymphoma, extracavitary primary effusion lymphoma, ALK+ DLBCL, and HHV8+ large B-cell lymphoma, among others. RISK STRATIFICATION: Age ≥60 years, advanced clinical stage, and high intermediate and high International Prognostic Index scores are associated with worse survival. MANAGEMENT: Combination chemotherapy regimens, such as EPOCH, are recommended. The addition of bortezomib, lenalidomide, or daratumumab might improve outcomes. Including PBL patients and their participation in prospective clinical trials is warranted.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica , Linfoma Plasmablástico , Humanos , Linfoma Plasmablástico/diagnóstico , Linfoma Plasmablástico/terapia , Linfoma Plasmablástico/tratamento farmacológico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Medição de Risco , Ciclofosfamida/uso terapêutico , Doxorrubicina/uso terapêutico , Doxorrubicina/administração & dosagem , Vincristina/uso terapêutico , Vincristina/administração & dosagem , Prednisona/uso terapêutico , Lenalidomida/uso terapêutico , Lenalidomida/administração & dosagem , Prognóstico , Bortezomib/uso terapêutico , Bortezomib/administração & dosagem , Diagnóstico Diferencial , Gerenciamento Clínico , Pessoa de Meia-Idade , Linfoma Difuso de Grandes Células B/diagnóstico , Linfoma Difuso de Grandes Células B/terapia , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Linfoma Difuso de Grandes Células B/genética , Anticorpos Monoclonais , EtoposídeoRESUMO
Plasmablastic lymphoma (PBL) is an aggressive non-Hodgkin lymphoma associated with HIV infection and immunodeficiency. However, PBL can also be seen immunocompetent individuals in recent studies. PBL was characterized by distinct clinical and pathological features, such as plasmablastic morphology and universal expression of plasma cell markers. The clinicopathologic features were different between HIV-negative and HIV-positive patients. Gene expression analysis identified the unique molecular feature in PBL, including frequent c-MYC rearrangement and downregulation of BCR signaling pathway. Despite the recent advances in the treatment of PBL, the prognosis of PBL patients remains dismal. The objectives of this review are to summarize the current knowledge on the epidemiology, molecular profiles, clinical and pathological features, differential diagnosis, treatment strategies, prognostic factors, and potential novel therapeutic approaches in PBL patients.
Assuntos
Infecções por HIV , Soropositividade para HIV , Linfoma Plasmablástico , Humanos , Linfoma Plasmablástico/diagnóstico , Linfoma Plasmablástico/genética , Linfoma Plasmablástico/terapia , Infecções por HIV/epidemiologia , Infecções por HIV/complicações , Prognóstico , Plasmócitos/patologiaRESUMO
BACKGROUND: Plasmablastic Lymphoma (PBL) is a rare aggressive B-cell lymphoma that primarily affects immunocompromised individuals, including those living with HIV. Historically, survival estimates are dismal and range from 8 to 15 months. We aimed to evaluate epidemiologic characteristics, treatment patterns and survival trends on a national scale. PATIENTS AND METHODS: Patients diagnosed with PBL from 2010 to 2020 were identified in the National Cancer Database (NCDB) and in the Surveillance, Epidemiology, and End Results (SEER) program. Incidence rates were calculated using SEER. Demographic features, treatment characteristics, and overall survival (OS) were identified using the NCDB. RESULTS: We identified 1153 patients in the SEER database and 1822 patients in the NCDB. The incidence of PBL is 0.07 cases per 100,000 US population per year. PBL is more common in males (77%), and white patients (77%), with 50% of cases in patients with HIV. Patients who were treated with multiagent chemotherapy had a median OS of 58.6 months. On multivariate Cox regression, we found that HIV status did not have a significant impact on OS. Factors associated with worse OS included advancing age and stage. CONCLUSION: We present the largest study to date on PBL. Among treated patients, we described a median OS of 58.6 months, greatly improved from previously reported estimates. We found that HIV status did not have a significant impact on OS. While OS remains poor, therapeutic advances over the last decade are promising and highlight the need for continued clinical advances aimed at improving therapeutic options for this rare lymphoma.
Assuntos
Infecções por HIV , Linfoma de Células B , Linfoma Plasmablástico , Masculino , Humanos , Estados Unidos/epidemiologia , Linfoma Plasmablástico/epidemiologia , Linfoma Plasmablástico/terapia , Análise de Sobrevida , Infecções por HIV/tratamento farmacológico , Infecções por HIV/epidemiologia , Programa de SEER , PrognósticoRESUMO
BACKGROUND: Plasmablastic lymphoma is a rare type of non-Hodgkin lymphoma that generally presents an aggressive clinical course. It is strongly associated with human immunodeficency virus (HIV) infection, and the most common site of involvement is the oral cavity. Although extraoral PBL has been reported in several places, small intestine involvement is extremely rare. CASE PRESENTATION: Here, we describe an exceptionally rare case of a 24-year-old immunocompetent Asian Male patient with newly diagnosed plasmablastic lymphoma of the duodenum. The patient was admitted to our oncology facility due to the patient's clinical course, which included persistent vomiting, hematemesis, weight loss, and generalized weakness. Computed tomography of the abdomen (triphasic) of the patient showed thickness at the 2nd part of the duodenum measuring 2.6 cm in width and 16 cm in length blocking the pancreatic and common bile ducts by entering the second section of the duodenum. The biopsy specimen's pathological investigation indicated abnormal cells with plasmacytoid characteristics and a high proliferation index. The diagnosis of PBL was confirmed by immunohistochemical profiling. Supportive therapies like blood transfusions, antacids, and antiemetics were started to manage the patient's symptoms. Palliative radiation was also anticipated for the lesion site. CONCLUSIONS: Duodenal involvement to the extent seen in our patient is exceptionally rare and, to the best of our knowledge, has hardly been described. The main goal of the article is to review the literature and report a case.
Assuntos
Linfoma Imunoblástico de Células Grandes , Linfoma não Hodgkin , Linfoma Plasmablástico , Humanos , Masculino , Adulto Jovem , Adulto , Linfoma Plasmablástico/complicações , Linfoma Plasmablástico/diagnóstico , Linfoma Plasmablástico/terapia , Linfoma Imunoblástico de Células Grandes/patologia , Duodeno/diagnóstico por imagem , Duodeno/patologia , Progressão da DoençaRESUMO
Plasmablastic Lymphoma is a rare large B-cell lymphoma with unique immunohistochemical and morphological features. It was most commonly associated with HIV infection; however, it's now seen in other immunosuppressed states like autoimmune conditions, post-transplant settings, and even in elderly immunocompetent individuals. Although rare, it is an aggressive lymphoma with unfavorable outcomes. The aim of this manuscript is to have an in-depth review of the current knowledge of epidemiology, pathophysiology, prognostic markers, and treatment approaches currently in use and in clinical trials for this challenging disease.
Assuntos
Infecções por HIV , Linfoma Difuso de Grandes Células B , Linfoma Plasmablástico , Humanos , Idoso , Linfoma Plasmablástico/diagnóstico , Linfoma Plasmablástico/terapia , Infecções por HIV/epidemiologia , Infecções por HIV/complicações , Linfoma Difuso de Grandes Células B/tratamento farmacológicoRESUMO
Plasmablastic lymphoma (PBL) is a rare and aggressive subtype of non-Hodgkin lymphoma that shares features with diffuse large B-cell lymphoma (DLBCL). While significant progress has been made in treating DLBCL, the prognosis for PBL remains poor, highlighting the need to identify new therapeutic targets. Using RNA expression analysis, we compared the expression of genes involved in the phosphatidylinositol-3-kinase (PI3K) and mitogen-activated protein kinase (MAPK) signaling pathways between PBL and DLBCL. We used critical PI3K (n = 201) and MAPK (n = 57) signaling probe sets to achieve this objective. Our results demonstrate unique molecular mechanisms underlying PBL pathogenesis compared to DLBCL, particularly within the PI3K and MAPK signaling pathways. We found that elevated STAT3 expression in PBL correlates with hyperactive MAPK and PI3K pathways, unlike DLBCL. Additionally, the hyperactivation of the PI3K signaling axis in PBL is unrelated to B-cell receptor or phosphatase and tensin homolog activity, indicating a distinct mechanism compared to DLBCL. Furthermore, we observed unique activation patterns in MAPK pathways between PBL and DLBCL, with PBL exhibiting high expression of the neurotrophic tyrosine kinase receptor (NTKR) family, specifically NTRK1 and NTRK2 genes, which have therapeutic potential. We also found that neither human immunodeficiency virus nor Epstein-Barr virus infection influences gene expression profiles linked to PI3K and MAPK signaling in PBL. These findings could lead to adapting targeted therapies developed for DLBCL to address the specific needs of PBL patients better and contribute to developing novel, targeted therapeutic strategies to improve patient outcomes.
Assuntos
Infecções por Vírus Epstein-Barr , Linfoma Difuso de Grandes Células B , Linfoma Plasmablástico , Transdução de Sinais , Humanos , Herpesvirus Humano 4 , Linfoma Difuso de Grandes Células B/terapia , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Fosfatidilinositol 3-Quinases/genética , Fosfatidilinositol 3-Quinases/metabolismo , Linfoma Plasmablástico/genética , Linfoma Plasmablástico/terapiaRESUMO
Plasmablastic lymphoma (PBL) is a rare entity, commonly associated with immunosuppressed states such as human immunodeficiency virus (HIV) infection or solid organ transplant. The clinical course is characterized by high relapse rates and a poor prognosis, leading some clinicians to recommend aggressive frontline therapy. However, a specific review of limited stage (LS) PBL patients is not available to evaluate outcomes and justify treatment recommendations. We performed a retrospective review of LS PBL cases to provide insight into this rare disease. Our cohort consisted of 80 stage I or II PBL patients from 13 US academic centers. With a median follow up of 34 months (1-196), the 3-year progression-free survival (PFS) and overall survival (OS) of the entire cohort were 72% (95% CI 62, 83) and 79% (95% CI 70, 89), respectively. The 3-year PFS and OS of patients treated with frontline chemotherapy alone was 65% (95% CI 50, 84) and 71% (95% CI 56, 89), respectively, compared to 85% (95% CI 72, 100) and 96% (95% CI 89, 100), respectively, in patients treated with combined frontline chemotherapy with radiation consolidation. Our data demonstrate favorable outcomes in LS PBL with no improvements in outcome from aggressive frontline treatment including Hyper-CVAD or auto-SCT consolidation. Multivariate regression analysis (MRA) demonstrated improved PFS for patients receiving EPOCH based frontline therapy versus CHOP (HR: 0.23; p = 0.029). Frontline chemotherapy followed by radiation consolidation versus chemotherapy alone appeared to be associated with improved relapse and survival outcomes but did not show statistical significance in MRA.
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Infecções por HIV , Linfoma Plasmablástico , Humanos , Linfoma Plasmablástico/terapia , Linfoma Plasmablástico/patologia , Estudos Retrospectivos , Recidiva Local de Neoplasia/etiologia , Protocolos de Quimioterapia Combinada Antineoplásica , Intervalo Livre de Progressão , Infecções por HIV/tratamento farmacológico , PrognósticoRESUMO
Plasmablastic lymphoma (PBL) represents a rare distinct lymphoma entity with plasmablastic morphology and plasmacytic immunophenotype that is characterized by an aggressive clinical course. Standard chemotherapeutic regimens often remain insufficient to cure affected patients. Recently, comprehensive molecular analyses of large cohorts of primary PBL samples have revealed the mutational landscape as well as the pattern of copy number alterations of this rare lymphoma subtype. Identification of recurrent aberrations affecting the JAK-STAT, RAS-RAF, NOTCH, IRF4, and MYC signaling pathways drive the molecular pathogenesis of PBL and hold great potential for novel targeted therapeutic approaches.
Assuntos
Linfoma Plasmablástico , Humanos , Linfoma Plasmablástico/diagnóstico , Linfoma Plasmablástico/genética , Linfoma Plasmablástico/terapia , Proteínas Proto-Oncogênicas c-myc/genética , Transdução de Sinais , Plasmócitos/patologiaRESUMO
Plasmablastic lymphoma (PBL) is a highly aggressive B cell non-Hodgkin lymphoma frequently associated with immunosuppression, particularly human immunodeficiency virus (HIV) infection. Although PBL is rare globally, South Africa has a high burden of HIV infection leading to a higher incidence of PBL in the region. Laboratory features in PBL may overlap with plasmablastic myeloma and other large B cell lymphomas with plasmablastic or immunoblastic morphology leading to diagnostic dilemmas. There are, however, pertinent distinguishing laboratory features in PBL such as a plasma cell immunophenotype with MYC overexpression, expression of Epstein-Barr virus-encoded small RNAs and lack of anaplastic lymphoma kinase (ALK) expression. This review aims to provide a summary of current knowledge in PBL, focusing on the epidemiology, pathophysiology, laboratory diagnosis and clinical management.
Assuntos
Infecções por Vírus Epstein-Barr , Infecções por HIV , Linfoma Plasmablástico , Infecções por Vírus Epstein-Barr/complicações , Infecções por Vírus Epstein-Barr/patologia , Infecções por HIV/complicações , Herpesvirus Humano 4 , Humanos , Imunofenotipagem , Linfoma Plasmablástico/diagnóstico , Linfoma Plasmablástico/epidemiologia , Linfoma Plasmablástico/terapiaRESUMO
The patient is a 34-year-old HIV antibody-negative female with normal immunocompetence. The patient was referred to the hospital of the current study due to diarrhea and abdominal pain, which developed in May 2014. On conducting computed tomography (CT), remarkable wall thickening was noted in the terminal ilium over the ascending colon, suggesting a malignant tumor. However, making a definite diagnosis by lower gastrointestinal endoscopic biopsy and left hemicolectomy was not possible. The dense proliferation of plasma cell-like cells and plasmablasts was noted; CD20, CD19, CD79a, CD3, CD4, and Epstein-Barr virus-encoded miRNAs (EBER) were negative and CD138 was positive on immunostaining. Based on the aforementioned data, the patient was diagnosed with plasmablastic lymphoma (PBL). High-dose chemotherapy combined with autologous peripheral blood stem cell transplantation (PBSCT) was performed in the first remission period after the completion of four cycles of hyper CVAD/MTX-AraC alternating therapy. Remission was confirmed by FDG-PET/CT 3 months after autologous PBSCT. No signs of recurrence have been observed in 6 years after the transplantation. Although no standard treatment for PBL has been established, autologous peripheral blood stem cell transplantation combined with high-dose chemotherapy during the first remission period may be a beneficial treatment option.
Assuntos
Infecções por Vírus Epstein-Barr , Infecções por HIV , Transplante de Células-Tronco de Sangue Periférico , Linfoma Plasmablástico , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica , Feminino , Infecções por HIV/complicações , Herpesvirus Humano 4 , Humanos , Linfoma Plasmablástico/diagnóstico , Linfoma Plasmablástico/terapia , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Transplante AutólogoRESUMO
Plasmablastic lymphoma (PBL) represents a rare and aggressive lymphoma subtype frequently associated with immunosuppression. Clinically, patients with PBL are characterized by poor outcome. The current understanding of the molecular pathogenesis is limited. A hallmark of PBL represents its plasmacytic differentiation with loss of B-cell markers and, in 60% of cases, its association with Epstein-Barr virus (EBV). Roughly 50% of PBLs harbor a MYC translocation. Here, we provide a comprehensive integrated genomic analysis using whole exome sequencing (WES) and genome-wide copy number determination in a large cohort of 96 primary PBL samples. We identify alterations activating the RAS-RAF, JAK-STAT, and NOTCH pathways as well as frequent high-level amplifications in MCL1 and IRF4. The functional impact of these alterations is assessed using an unbiased shRNA screen in a PBL model. These analyses identify the IRF4 and JAK-STAT pathways as promising molecular targets to improve outcome of PBL patients.
Assuntos
Linfoma Plasmablástico/genética , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Estudos de Coortes , Feminino , Amplificação de Genes , Dosagem de Genes , Perfilação da Expressão Gênica , Humanos , Fatores Reguladores de Interferon/genética , Fatores Reguladores de Interferon/metabolismo , Janus Quinases/genética , Janus Quinases/metabolismo , Masculino , Pessoa de Meia-Idade , Terapia de Alvo Molecular , Linfoma Plasmablástico/metabolismo , Linfoma Plasmablástico/mortalidade , Linfoma Plasmablástico/terapia , Fatores de Transcrição STAT/genética , Fatores de Transcrição STAT/metabolismo , Translocação Genética , Sequenciamento do Exoma , Adulto JovemRESUMO
INTRODUCTION: No standard guideline has been established for the treatment of plasmablastic lymphoma (PBL) and prognosis remains extremely poor, given that patients relapse early after chemotherapy and show resistance to commonly used cytostatic drugs. PATIENT CONCERNS: We present the case of a 52-year-old HIV-negative man who presented with a mass at the left sternoclavicular joint. He had no significant comorbidities and no latent immunosuppression. DIAGNOSIS: The largest lymph node measured was 36â×â19âmm. An excisional biopsy showed diffuse proliferation of large lymphoid cells which were positive for CD38 and CD138, but negative for CD20. He was diagnosed with stage IV PBL with a low IPI. INTERVENTIONS: The patient was treated with four cycles of induction therapy with bortezomib, epirubicin and dexamethasone. He achieved complete remission. But 3âmonths after receiving consolidated autologous hematopoietic stem cell transplantation, he relapsed. Allogeneic hematopoietic stem cell transplantation was performed on the patient. OUTCOMES: The patient achieved remission again and there were no serious complications after allogeneic hematopoietic stem cell transplantation. This patient was followed up once every three months, and to date, he has been disease-free for more than 4âyears. CONCLUSION: The survival of recurrent PBL after autologous hematopoietic stem cell transplantation is very poor. Salvage allogeneic hematopoietic stem cell transplantation may bring long-term survival opportunities for those patients. Further clinical studies are needed to explore the role of allogeneic hematopoietic stem cell transplantation in refractory and recurrent PBL.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Transplante de Células-Tronco Hematopoéticas/métodos , Linfoma Plasmablástico/terapia , Transplante Homólogo/métodos , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Soronegatividade para HIV , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Humanos , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/terapia , Linfoma Plasmablástico/diagnóstico , Linfoma Plasmablástico/patologia , Indução de Remissão/métodos , Transplante Homólogo/efeitos adversosRESUMO
Plasmablastic lymphoma (PBL) is a newly recognized aggressive subtype of non-Hodgkin lymphoma. Its rarity hinders testing effective treatment options in clinical trials. We conducted a systematic review of PubMed and our internal records to retrieve patients with a PBL diagnosis with evaluable treatment outcomes. Aggressive chemotherapy was defined as more intense regimens than CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone). We compiled a meta-dataset of 173 patients. The median age at diagnosis was 48.5 years, 75% of patients were male, and stages III/IV accounted for 47% of the cohort. Of 138 patients with known response status after first-line chemotherapy, 63 (45%) achieved a complete response with a 2-year relapse-free survival of 71.6%. Sixty-nine (50%) patients received first-line CHOP. There was no significant difference in the objective response rate among the 2 most commonly used regimens, CHOP and DA-EPOCH (dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin) (69% vs. 79%; P = .4). The median follow-up was 9 months, and the 2-year overall survival (OS) was 47.4%. A univariate analysis identified factors associated with worse OS, including stage III/IV (hazard ratio [HR], 2.82; P < .001), human herpes virus-8-positive (HR, 3.30; P = .01), bone marrow (HR, 1.07; P = .035), and cardiorespiratory involvement (HR, 2.26; P = .015). Meanwhile, Epstein-Varr virus-encoded small RNA-positivity (HR, 0.31; P < .001) and involvement of head and neck (HR, 0.44; P = .009) were associated with better OS. Multivariate analysis showed that aggressive chemotherapy was significantly associated with better OS (HR, 0.22; P = .016). Patients with PBL with high-risk features, such as advanced stage, human herpes virus-8-positivity, bone marrow, and cardiorespiratory involvement, require more aggressive chemotherapy. Bortezomib and lenalidomide are promising add-on agents.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Linfoma Plasmablástico/mortalidade , Linfoma Plasmablástico/terapia , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Tomada de Decisão Clínica , Comorbidade , Gerenciamento Clínico , Suscetibilidade a Doenças , Perfilação da Expressão Gênica , Humanos , Estimativa de Kaplan-Meier , Linfoma Plasmablástico/diagnóstico , Linfoma Plasmablástico/etiologia , Prognóstico , Modelos de Riscos Proporcionais , Resultado do TratamentoRESUMO
Activated phosphoinositide 3-kinase δ syndrome (APDS) is an autosomal dominant primary immunodeficiency caused by acquired gene function mutation (GOF). APDS has a variety of clinical phenotypes, particularly recurrent respiratory infections and lymphoproliferation. Here we report a pediatric patient with APDS who presented with recurrent respiratory infections, lymphoproliferation, hepatosplenomegaly, bronchoscopy suggesting numerous nodular protrusions in the airways and a decrease in both T and B lymphocytes, and progression to plasmablastic lymphoma (PBL) after 1 year. Whole exome sequencing revealed a heterozygous mutation in the PIK3CD gene (c.3061 G>A p.E1021K). This is the first reported case of APDS combined with PBL and pediatricians should follow up patients with APDS regularly to be alert for secondary tumours.
Assuntos
Linfoma Plasmablástico/imunologia , Doenças da Imunodeficiência Primária/complicações , Pré-Escolar , Classe I de Fosfatidilinositol 3-Quinases , Feminino , Transplante de Células-Tronco Hematopoéticas/métodos , Humanos , Linfoma Plasmablástico/terapiaRESUMO
Plasmablastic lymphoma is an aggressive variant of lymphomas recently distinct from diffuse large B cell lymphoma. It has been initially described as a disease affecting the oral cavity of immunocompromised patients. We here report the first case of a 54-year-old patient with nasal septum nodule, bleeding on contact and after sneezing which occurred 6 months before admission. Facial computed tomography (CT) scan showed thickening of the nasal mucosa of 14mm. Excisional biopsy showed tumor proliferation composed of plasmablastic cells with immunophenotypic features: CD 138+, ki67 80%, EMA+, CD79a+, CD 56+. Staging and HIV serology were negative. Given the rarity of this lymphoma there are no standard treatments and most patients have treatment-resistant lymphoma with poor prognosis. Our patient received 6 cycles of CHOP-like chemotherapy associated with 40 gy radiotherapy in 20 fractions of 2 gy with complete remission (unusual in the cases described in the literature).
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Mucosa Nasal/patologia , Neoplasias Nasais/terapia , Linfoma Plasmablástico/terapia , Biópsia , Ciclofosfamida/administração & dosagem , Doxorrubicina/administração & dosagem , Etoposídeo/administração & dosagem , Humanos , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Neoplasias Nasais/patologia , Linfoma Plasmablástico/patologia , Prednisolona/administração & dosagem , Indução de Remissão , Tomografia Computadorizada por Raios X , Vincristina/administração & dosagemRESUMO
Herein we analyzed survival outcomes in chemotherapy-treated patients with plasmablastic lymphoma (PBL) diagnosed between 2010 to 2016 (n = 248). Data was acquired from the Surveillance, Epidemiology, and End Results (SEER) 18 registries database (April 2019 release based on November 2018 submission). The majority of patients were male (81.9%) and younger than 60 years (71.0%). Oral and gastrointestinal (GI) sites were the most frequent primary extranodal locations (23% and 19.4%, respectively). Oral primary location was inversely associated with presence of B symptoms and advanced Ann-Arbor stage. The 3-year and 5-year overall survival (OS) rates of treated PBL patients were 54% (95% CI: 46.5%-60.8%) and 52.8% (95% CI: 45.2%-59.8%). Three-year conditional survival for 2-year and 3-year survivors were 90.3% and 97.8%, overlapping the survival of a general population matched by age, sex and calendar year. In a multivariable analysis, oral primary location was associated with not only better OS (HR 0.43; 95% CI: 0.21-0.88, P = .021) but also better lymphoma-specific survival (LSS) (SHR 0.36; 95% CI: 0.15-0.86, P = .022); age ≥60 years was associated with shorter LSS (SHR 1.73; 95% CI: 1.02-2.96, P = .043). Seven registries granted access to HIV status (n = 93) where HIV infection was detected in 52.7% of cases. The HIV status did not affect survival outcomes in unadjusted and adjusted analyses. We identified clinical characteristics associated with survival and showed that treated PBL patients may achieve long-term survival.
Assuntos
Linfoma Plasmablástico/mortalidade , Sistema de Registros , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Intervalo Livre de Doença , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Linfoma Plasmablástico/terapia , Fatores de Risco , Taxa de Sobrevida , Estados Unidos/epidemiologiaAssuntos
Deleção Cromossômica , Mieloma Múltiplo/genética , Mieloma Múltiplo/terapia , Linfoma Plasmablástico/genética , Linfoma Plasmablástico/terapia , Síndrome de Smith-Magenis , Adulto , Biomarcadores Tumorais , Biópsia , Cromossomos Humanos Par 17 , Feminino , Humanos , Imuno-Histoquímica , Mieloma Múltiplo/diagnóstico , Linfoma Plasmablástico/diagnóstico , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Tomografia Computadorizada por Raios XRESUMO
We describe an exceptionally rare case of a male patient with newly diagnosed advanced human immunodeficiency virus (HIV) infection, who presented with a plasmablastic lymphoma involving the right maxillary alveolar ridge with associated cervical lymphadenopathy. On a staging positron emission tomography computed tomography (PET-CT) scan, he was incidentally found to have an endotracheal tumour involving the anterolateral aspect of the mid-trachea. The tumour appeared to be well-vascularised at bronchoscopy and was confirmed as well-differentiated plasmablastic lymphoma. Plasmablastic lymphoma is a rare form of non-Hodgkin lymphoma and is associated with HIV. Tracheal involvement to the extent seen in our patient is exceptionally rare, and, to the best of our knowledge, has never been described.