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Primary Mediastinal Large B-Cell Lymphoma

Large Cell Lymphoma of the Mediastinum


Presentation

  • The aim of this retrospective study was to investigate the frequency of gastric involvement in 204 consecutive patients with PMLBCL that presented at 23 hospitals in Greece. Two out of 204 patients (1.0%) had gastric involvement at presentation.[ncbi.nlm.nih.gov]
  • We report a case of primary mediastinal large B cell lymphoma in a 46-year-old woman of African descent who is human immunodeficiency virus positive who presented with symptoms of superior vena cava syndrome.[ncbi.nlm.nih.gov]
  • Some interlacing spindle cells and epithelioid cells were also present.[ncbi.nlm.nih.gov]
  • Patients were treated based on era-specific BCCA guidelines (1980-1992 MACOPB/VACOPB; 1992-2001 CHOP-type; 2001-present CHOP-R).[ncbi.nlm.nih.gov]
  • We report a case of an HIV-infected patient who presents with primary mediastinal large B-cell lymphoma.[ncbi.nlm.nih.gov]
Mediastinal Lymphadenopathy
  • CT scan revealed a 5-cm anterior mediastinal mass and mediastinal lymphadenopathy. Endoscopic ultrasound-guided FNA of a 4.5-cm subcarinal lymph node showed medium to large atypical lymphocytes with scant to moderate finely vacuolated cytoplasm.[ncbi.nlm.nih.gov]
Axillary Lymphadenopathy
  • A 57-yr-old woman presented with bilateral axillary lymphadenopathy and intermittent shortness of breath. CT scan revealed a 5-cm anterior mediastinal mass and mediastinal lymphadenopathy.[ncbi.nlm.nih.gov]
Fever
  • Systemic symptoms, mainly fever or weight loss, are present in[ncbi.nlm.nih.gov]
  • The patient, a 33-year-old man, had cough, dyspnea, fever, superior vena cava syndrome, and a mediastinal bulky tumor. A biopsy showed that the latter was characterized by large cells, sclerosis, and compartmentalization.[ncbi.nlm.nih.gov]
  • […] in the multicenter group (2.5 years follow-up).Short-term toxicity was manageable, but required hospitalization: the rates of grade 3 or 4 toxicity were 20% (for mucositis), 42% (for neutropenia), 29% (for thrombocytopenia), and 9% (for neutropenic fever[ncbi.nlm.nih.gov]
  • Systemic symptoms mainly fever and weight loss, were present in all cases; increased LDH levels were also observed in 50% of cases. Majority of the patients (83%) presented with stage I/II disease, bulky mass with a median tumor diameter 15 cm.[cancerres.aacrjournals.org]
Fatigue
  • The most common adverse reactions (reported in at least 20% of patients) were fatigue, decreased appetite, and dyspnea.[businesswire.com]
  • Common symptoms breathlessness* cough or chest pain* fatigue loss of appetite enlarged lymph nodes - painless swelling in the neck, armpit or groin - often in more than one group.[lymphomation.org]
Chills
  • Monitor patients for signs and symptoms of infusion-related reactions, including rigors, chills, wheezing, pruritus, flushing, rash, hypotension, hypoxemia, and fever. For Grade 3 or 4 reactions, stop infusion and permanently discontinue KEYTRUDA.[businesswire.com]
Cough
  • PMLBCL is characterized by a locally invasive anterior mediastinal mass, often producing cough, chest pain, dyspnea, and superior vena cava syndrome.[ncbi.nlm.nih.gov]
  • The patient, a 33-year-old man, had cough, dyspnea, fever, superior vena cava syndrome, and a mediastinal bulky tumor. A biopsy showed that the latter was characterized by large cells, sclerosis, and compartmentalization.[ncbi.nlm.nih.gov]
  • Also called thymus gland. superior vena cava syndrome A group of symptoms that include coughing, difficulty breathing, headache, dizziness, fainting and swelling or flushing of the neck, face and upper arms.[cancer.ca]
  • Primary mediastinal B-cell lymphoma often presents with symptoms of cough, shortness of breath, or swelling of the head and neck, due to the tumor pressing on the windpipe and the large veins above the heart.[danafarberbostonchildrens.org]
  • Patient 2, a 14-year-old boy, presented with swallowing discomfort and dry cough. Imaging study showed a huge mediastinal mass and pleural effusions.[iss.ndl.go.jp]
Pleural Effusion
  • The two groups of patients were similar as regard to age, gender, presence of bulky mediastinal mass, pleural effusion, stage and international prognostic indexes category of risk. Overall, 75.5% of patients in CR received IF-RT as consolidation.[ncbi.nlm.nih.gov]
  • Breathing problems, shortness of breath, pleural effusions, superior vena cava syndrome, fevers. etc.[nhlcyberfamily.org]
  • Imaging study showed a huge mediastinal mass and pleural effusions. In both patients, the tumor cells showed mature B cell phenotype, and histopathology in patient 2 showed diffuse large-cell lymphoma with sclerosis.[iss.ndl.go.jp]
  • The most frequent serious adverse reactions reported in at least 2% of patients were pneumonia, dyspnea, confusional state, vomiting, pleural effusion, and respiratory failure.[businesswire.com]
  • effusions ( Table 3 ).[haematologica.org]
Dyspnea
  • PMLBCL is characterized by a locally invasive anterior mediastinal mass, often producing cough, chest pain, dyspnea, and superior vena cava syndrome.[ncbi.nlm.nih.gov]
  • The patient, a 33-year-old man, had cough, dyspnea, fever, superior vena cava syndrome, and a mediastinal bulky tumor. A biopsy showed that the latter was characterized by large cells, sclerosis, and compartmentalization.[ncbi.nlm.nih.gov]
  • The most common adverse reactions (reported in at least 20% of patients) were fatigue, decreased appetite, and dyspnea.[businesswire.com]
Tracheal Deviation
  • A chest X-ray revealed a homogenous opacification on her right side with a left-sided tracheal deviation while a computed tomography scan of her chest revealed a solid mass on her right side.[ncbi.nlm.nih.gov]
Loss of Appetite
  • Common symptoms breathlessness* cough or chest pain* fatigue loss of appetite enlarged lymph nodes - painless swelling in the neck, armpit or groin - often in more than one group.[lymphomation.org]
Dysphagia
  • […] the pressure of the mass on the lungs or gut, or on the superior vena cava (the second largest vein in the body which returns blood from the upper half of the body to the heart).Symptoms include breathlessness, persistent cough, difficulty swallowing (dysphagia[leukaemia.org.au]
Chest Pain
  • PMLBCL is characterized by a locally invasive anterior mediastinal mass, often producing cough, chest pain, dyspnea, and superior vena cava syndrome.[ncbi.nlm.nih.gov]
  • The mass can cause symptoms as it grows bigger, such as: shortness of breath cough chest pain a partial blockage of the main vein that carries blood from the upper body to the heart (called the superior vena cava), which can lead to superior vena cava[cancer.ca]
  • Subsequently, her chest pain got worse, and she went to another clinic, where she was detected with an anterior mediastinal tumor and was referred to our hospital. Tumor biopsy with a thoracoscope was performed, and a diagnosis of PMBL was made.[dear-medic.jp]
  • Common symptoms breathlessness* cough or chest pain* fatigue loss of appetite enlarged lymph nodes - painless swelling in the neck, armpit or groin - often in more than one group.[lymphomation.org]

Workup

  • Workup of such cases should include extended panel of immunohistochemical stains including the B-cell transcription factors PAX-5, OCT-2 and BOB.1.[arup.utah.edu]
Pericardial Effusion
  • Our simple indicators of International Prognostic Index score and the presence of pleural or pericardial effusion could stratify patients with primary mediastinal large B-cell lymphoma and help guide selection of treatment.[ncbi.nlm.nih.gov]
  • Most PMLBCL patients have stage I-II, bulky disease, with pleural or pericardial effusions in a third of cases. Systemic symptoms, mainly fever or weight loss, are present in[ncbi.nlm.nih.gov]
  • Most had features of local extension like pleuro-pericardial effusion. Absence of infradiaphragmatic lymph nodes and no bone marrow involvement at presentation. All patients required biopsy from the mediastinal mass by image guided core biopsy.[cancerres.aacrjournals.org]
  • The second patient had a large mediastinal tumor, pleural and pericardial effusions as well as enlarged abdominal lymph nodes.[haematologica.org]
  • Focusing on patients treated with cyclophosphamide, doxorubicin, vincristine, prednisolone, and rituximab (R-CHOP) ( N 187), a higher International Prognostic Index (IPI) and the presence of pleural or pericardial effusion were identified as adverse prognostic[ci.nii.ac.jp]
Anterior Mediastinal Mass
  • PMLBCL is characterized by a locally invasive anterior mediastinal mass, often producing cough, chest pain, dyspnea, and superior vena cava syndrome.[ncbi.nlm.nih.gov]
  • CT scan revealed a 5-cm anterior mediastinal mass and mediastinal lymphadenopathy. Endoscopic ultrasound-guided FNA of a 4.5-cm subcarinal lymph node showed medium to large atypical lymphocytes with scant to moderate finely vacuolated cytoplasm.[ncbi.nlm.nih.gov]
  • Radiologically, there was a huge anterior mediastinal mass. Tru-cut biopsy was initially diagnosed as type-A thymoma.[ncbi.nlm.nih.gov]
  • mediastinal mass, and complications such as superior vena cava syndrome are common at presentation.[doi.org]
Multiple Pulmonary Nodules
  • Computed tomography of the chest and abdomen demonstrated a bulky mass in the left anterior mediastinum, multiple pulmonary nodules, axillary lymph node swelling, and a pancreatic tumor.[ncbi.nlm.nih.gov]
Pleural Effusion
  • The two groups of patients were similar as regard to age, gender, presence of bulky mediastinal mass, pleural effusion, stage and international prognostic indexes category of risk. Overall, 75.5% of patients in CR received IF-RT as consolidation.[ncbi.nlm.nih.gov]
  • Breathing problems, shortness of breath, pleural effusions, superior vena cava syndrome, fevers. etc.[nhlcyberfamily.org]
  • Imaging study showed a huge mediastinal mass and pleural effusions. In both patients, the tumor cells showed mature B cell phenotype, and histopathology in patient 2 showed diffuse large-cell lymphoma with sclerosis.[iss.ndl.go.jp]
  • The most frequent serious adverse reactions reported in at least 2% of patients were pneumonia, dyspnea, confusional state, vomiting, pleural effusion, and respiratory failure.[businesswire.com]
  • effusions ( Table 3 ).[haematologica.org]

Treatment

  • CONCLUSIONS: Our data indicate that a third-generation regimen like MACOP-B could be considered a suitable first-line treatment.[ncbi.nlm.nih.gov]
  • This article reviews the treatment approach for mediastinal lymphomas, including CHL of the nodular sclerosis subtype (CHL-NS), PMBL, and mediastinal GZL.[ncbi.nlm.nih.gov]
  • Planned treatment was concluded in 43 of 53 patients; in 10 patients, I-CT was not immediately followed by IFRT.[ncbi.nlm.nih.gov]
  • Treatment options for relapsed/refractory primary mediastinal large B-cell lymphoma (rrPMBCL) are limited, and prognosis is generally poor (overall response rate [ORR] 0% to 25%; 2-year overall survival 15%).[ncbi.nlm.nih.gov]
  • Therefore, the need for more aggressive treatment strategies is questionable unless high-risk patients are adequately defined. Further studies are required to establish the precise role of RT.[ncbi.nlm.nih.gov]

Prognosis

  • The prognosis and therapy of patients with PMLBCL is still controversial. We summarize our experience with PMLBCL at the Medical University of South Carolina between 1997 and 2000.[ncbi.nlm.nih.gov]
  • These data are consistent with those reported from other centers concerning the patient's characteristics, natural history, response pattern to chemoradiation therapy, and prognosis. Response to therapy proved of prognostic significance.[ncbi.nlm.nih.gov]
  • For this group of patients with homogeneous clinical characteristics, response to initial chemotherapy and MUM1 expression were associated with prognosis.[ncbi.nlm.nih.gov]
  • The estimated 3-year overall and failure-free survival rates were 32% and 33%, respectively, indicating the relatively unfavorable prognosis of the patients in our series.[ncbi.nlm.nih.gov]
  • Treatment options for relapsed/refractory primary mediastinal large B-cell lymphoma (rrPMBCL) are limited, and prognosis is generally poor (overall response rate [ORR] 0% to 25%; 2-year overall survival 15%).[ncbi.nlm.nih.gov]

Etiology

  • For suspected immune-mediated adverse reactions, ensure adequate evaluation to confirm etiology or exclude other causes. Based on the severity of the adverse reaction, withhold KEYTRUDA (pembrolizumab) and administer corticosteroids.[businesswire.com]
  • The use of tocilizumab directed at IL-6 has been shown to mitigate CRS, but the etiology and treatment of neurologic complications remain more elusive.[hematology.org]
  • Implications for etiology and pathogenesis. Cancer. 71 : 2062 –2071. Addis, B.J., and P.G. Isaacson. 1986. Large cell lymphoma of the mediastinum: a B-cell tumour of probable thymic origin. Histopathology. 10 : 379 –390. Isaacson, P.G., A.J.[jem.rupress.org]

Epidemiology

  • Gov't MeSH terms B7-H1 Antigen/genetics* Cell Line, Tumor Chromosomes, Human, Pair 9 DNA Copy Number Variations Gene Expression Regulation, Neoplastic Gene Frequency Humans In Situ Hybridization, Fluorescence Lymphoma, Large B-Cell, Diffuse/epidemiology[ncbi.nlm.nih.gov]
  • We used Surveillance, Epidemiology and End Results (SEER) 18 database to compare the overall survival (OS) differences among adult patients treated with and without RT after rituximab approval in the US.[ncbi.nlm.nih.gov]
  • Abstract The aim of this study is to investigate the incidence and clinical outcomes of primary mediastinal large B-cell lymphoma (PMBL).Here we did a retrospective analysis using the surveillance, epidemiology, and end results (SEER) database to analyze[ncbi.nlm.nih.gov]
  • Chemotherapy Protocols/therapeutic use Asian Continental Ancestry Group Child Disease-Free Survival Female Humans Incidence Lymphoma, B-Cell Lymphoma, Large B-Cell, Diffuse/diagnosis Lymphoma, Large B-Cell, Diffuse/drug therapy Lymphoma, Large B-Cell, Diffuse/epidemiology[ncbi.nlm.nih.gov]
  • : navigation, search Primary mediastinal large B-cell lymphoma Microchapters Home Patient Information Overview Historical Perspective Classification Pathophysiology Causes Differentiating Primary Mediastinal Large B-cell Lymphoma from other Diseases Epidemiology[wikidoc.org]
Sex distribution
Age distribution

Pathophysiology

  • (Redirected from Mediastinal large B-cell lymphoma ) Jump to: navigation, search Primary mediastinal large B-cell lymphoma Microchapters Home Patient Information Overview Historical Perspective Classification Pathophysiology Causes Differentiating Primary[wikidoc.org]

Prevention

  • This case suggests that long-term administration of an LMWH contributes to the primary improvement and secondary prevention of VTE even in patients who are at high risk for thrombosis.[ncbi.nlm.nih.gov]
  • Secondary Prevention Cost-Effectiveness of Therapy Future or Investigational Therapies Case Studies Case #1 Primary mediastinal large B-cell lymphoma On the Web Most recent articles Most cited articles Review articles CME Programs Powerpoint slides Images[wikidoc.org]
  • Clinical trials that integrate novel therapies into upfront immunochemotherapy and utilize end-of-therapy PET scan to guide mediastinal radiation have potential to further enhance survival and prevent long-term toxicities.[ncbi.nlm.nih.gov]
  • Your doctor may check the fluid around the brain, called cerebrospinal fluid (CSF), in certain cases at diagnosis and recommend treatments to prevent the lymphoma from spreading to the brain, called central nervous system (CNS) prophylaxis.[cancer.net]
  • In vitro, neutralizing antibodies directed against CD2 or CD58 prevents conjugate formation and decreases T‐cell activity (Mak & Saunders, 2006 ).[doi.org]

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