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Primary Mediastinal Large B-Cell Lymphoma
Large Cell Lymphoma of the Mediastinum

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WIKIDATA, CC BY-SA 3.0

Presentation

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]

This lymphoma affects mostly young women, presenting mostly between the third and fourth decade of life. It presents as a rapidly growing mass in the anterior mediastinum, during its evolution it infiltrates adjacent structures. [revistadehematologia.org.mx]

Bone marrow or distant lymph node involvement at presentation is unusual [4]. [hindawi.com]

Entire Body System

  • Fever

    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 [hal.archives-ouvertes.fr]

  • Fatigue

    Clinical History A 15-year-old male patient presented with a 3-months history of shortness of breath, decreased appetite, fatigue and fever. A chest radiograph revealed a massive left-sided pleural effusion. [eurorad.org]

    The most common adverse reactions (reported in at least 20% of patients) were fatigue, decreased appetite, and dyspnea. [businesswire.com]

    She also experienced fatigue, prominent facial swelling, engorged neck vasculature, headaches, and a 25-pound weight loss. Climbing even four individual stairs caused this former soccer athlete significant fatigue and lightheadedness. [hindawi.com]

    The cause of the condition is also unknown, though genetic mutations have been implicated The condition can cause chest pain, breathing difficulties, fatigue, and other general signs and symptoms, such as fever, weight loss, and appetite loss. [dovemed.com]

  • Chills

    She denied having fevers, chills, or night sweats. She is a full-time college student living with her parents and sibling in Mexico. She was evaluated there and diagnosed with Cushing syndrome and hypothyroidism and was prescribed levothyroxine. [hindawi.com]

    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]

  • Cushingoid

    The swelling was so marked that it was initially interpreted as Cushingoid. In a young female, a large anterior mediastinal mass is most likely lymphoma, thymoma, germ cell tumor, or sarcoma. [hindawi.com]

Respiratoric

  • Cough

    PMLBCL is characterized by a locally invasive anterior mediastinal mass, often producing cough, chest pain, dyspnea, and superior vena cava syndrome. [moh-it.pure.elsevier.com]

    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]

    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]

  • Pleural Effusion

    Poor prognostic indicators are 1,2: advanced stage at diagnosis direct involvement of thoracic viscera infra-diaphragmatic spread pleural effusion Complications SVC syndrome respiratory compromise due to tracheal compression pleural effusions The differential [radiopaedia.org]

    Other features include parenchymal invasion, pleural effusion, pericardial effusion, chest wall invasion, tracheal, oesophageal and vascular compression. [eurorad.org]

    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]

    The disease typically presents with a bulky mediastinal mass and can be associated with pleural and pericardial effusions. [hematologyandoncology.net]

  • Dyspnea

    PMLBCL is characterized by a locally invasive anterior mediastinal mass, often producing cough, chest pain, dyspnea, and superior vena cava syndrome. [moh-it.pure.elsevier.com]

    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]

    […] usually presents with a bulky and rapidly expanding anterior mediastinal mass, often with pleural and pericardial effusions, and that can invade the lungs, superior vena cava, pleura, pericardium, and chest wall, leading to manifestations of cough, dyspnea [orpha.net]

    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]

    A respiratory examination revealed reduced chest expansion, stony dull percussion note, and absent breath sounds on her entire right side with a left-sided tracheal deviation. [jmedicalcasereports.biomedcentral.com]

Gastrointestinal

  • 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]

    The cause of the condition is also unknown, though genetic mutations have been implicated The condition can cause chest pain, breathing difficulties, fatigue, and other general signs and symptoms, such as fever, weight loss, and appetite loss. [dovemed.com]

    Around 1 in 3 people with DLBCL experience fevers, night sweats and unexplained weight loss. These are known as ‘B symptoms’. Fatigue and loss of appetite are also quite common, and some people experience severe itching. [lymphoma-action.org.uk]

  • Regurgitation

    This particular patient’s case was interesting because of the clear infiltration through the cardiac wall and invasion of the right atrium leading to tricuspid regurgitation. [hindawi.com]

    An apex beat was felt at the ninth intercostal space along the mid clavicular line and auscultation revealed a murmur of tricuspid regurgitation. A local breast examination revealed an edematous right breast that was otherwise normal. [jmedicalcasereports.biomedcentral.com]

  • 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]

Cardiovascular

  • Chest Pain

    PMLBCL is characterized by a locally invasive anterior mediastinal mass, often producing cough, chest pain, dyspnea, and superior vena cava syndrome. [moh-it.pure.elsevier.com]

    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]

    PMBCL is a large, fast growing tumor that causes symptoms of cough, rapid breathing, difficulty in swallowing and chest pain which are due to the presence of the tumor mass and resulting compression of surrounding tissues. [drugs.com]

  • Diastolic Murmur

    A faint diastolic murmur was heard best over the right sternal border. No lymphadenopathy was noted. [hindawi.com]

Face, Head & Neck

  • Collar of Stokes

    In addition, cardiac MRI and CT scan of the chest revealed the large mediastinal mass completely surrounding and eroding into the superior vena cava (SVC) wall causing a collar of stokes. [hindawi.com]

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]

X-Ray

  • 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. [moh-it.pure.elsevier.com]

    Other features include parenchymal invasion, pleural effusion, pericardial effusion, chest wall invasion, tracheal, oesophageal and vascular compression. [eurorad.org]

    The presence of pleural or pericardial effusions has been proposed as a negative prognostic indicator. [connect.springerpub.com]

  • Nodular Fibrosis

    A CT-guided biopsy of the mass revealed atypical large lymphoid cells with prominent nucleoli, abundant cytoplasm, and nodular fibrosis. [hindawi.com]

Pleura

  • Pleural Effusion

    Poor prognostic indicators are 1,2: advanced stage at diagnosis direct involvement of thoracic viscera infra-diaphragmatic spread pleural effusion Complications SVC syndrome respiratory compromise due to tracheal compression pleural effusions The differential [radiopaedia.org]

    Other features include parenchymal invasion, pleural effusion, pericardial effusion, chest wall invasion, tracheal, oesophageal and vascular compression. [eurorad.org]

    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]

    The disease typically presents with a bulky mediastinal mass and can be associated with pleural and pericardial effusions. [hematologyandoncology.net]

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]

You may have some treatments as part of a clinical trial. PMBCL usually responds well to treatment. But if it does not respond or if the lymphoma comes back, you will need more treatment. [macmillan.org.uk]

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]

[…] abbreviated PMBL, is a rare type of lymphoma that forms in the mediastinum (the space in between the lungs) and predominantly affects young adults.[1][2] It is a subtype of diffuse large B-cell lymphoma;[3] however, it generally has a significantly better prognosis [en.wikipedia.org]

Patient Information Overview Historical Perspective Classification Pathophysiology Causes Differentiating Primary Mediastinal Large B-cell Lymphoma from other Diseases Epidemiology and Demographics Risk Factors Screening Natural History, Complications and Prognosis [wikidoc.org]

H&O What is the prognosis? RA Overall, the prognosis is good, with a high response rate. Approximately 90% of patients are cured with contemporary therapies. [hematologyandoncology.net]

Etiology

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]

[…] unspecified site ICD-10: C85.29 - mediastinal (thymic) large B cell lymphoma, extranodal and solid organ sites Sites Presents with localized anterosuperior mediastinal mass arising in thymic area Regional supraclavicular and cervical lymph node involvement Etiology [pathologyoutlines.com]

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]

(Etiology) Lymphocytes are a type of white blood cells that are responsible for providing immunity in the human body. B-cells and T-cells are the two different types of lymphocytes. [dovemed.com]

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]

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]

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]

[…] diffuse large B-cell lymphoma involving the mediastinum, recognized in the WHO 2008 classification.[1][2]:370–374 Signs and symptoms[edit] Superior vena cava syndrome occurs in 30–50%, and pleural or pericardial effusions occur in about one-third.[3] Pathophysiology [en.wikipedia.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]

How can Primary Mediastinal Large B-Cell Lymphoma be Prevented? Currently, it is not possible to prevent Primary Mediastinal Large B-Cell Lymphoma. However, controlling certain factors may help lower one’s risk for the condition. [dovemed.com]

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