Diffuse Large B-Cell Lymphoma-Associated with Chronic Inflammation

Diffuse Large B-Cell Lymphoma-Associated with Chronic Inflammation

Article
Healthy Aging
Diseases & Conditions
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Contributed bySumanth Marupuru+1 moreJul 11, 2024

What are the other Names for this Condition? (Also known as/Synonyms)

  • Chronic Inflammation-Associated Diffuse Large B-Cell Lymphoma
  • DLBCL-Associated with Chronic Inflammation
  • DLBCL-CI (Diffuse Large B-Cell Lymphoma-Associated with Chronic Inflammation)

What is Diffuse Large B-Cell Lymphoma-Associated with Chronic Inflammation? (Definition/Background Information)

  • Diffuse Large B-Cell Lymphoma-Associated with Chronic Inflammation (DLBCL-CI) is a subtype of diffuse large B-cell lymphoma (DLBCL) that arises in the setting of long-standing inflammation or chronic infection. This type of lymphoma is closely associated with inflammatory conditions, such as autoimmune diseases, chronic infections, or other inflammatory disorders
  • Chronic inflammation can arise from various causes, such as autoimmune diseases, chronic infections, or inflammatory conditions like rheumatoid arthritis or inflammatory bowel disease. The presence of chronic inflammation in the tissue microenvironment may contribute to the development and progression of DLBCL in these cases. Understanding the association between chronic inflammation and DLBCL is essential for developing targeted treatment strategies and improving patient outcomes
  • The signs and symptoms of Diffuse Large B-Cell Lymphoma-Associated with Chronic Inflammation may include painless swelling of lymph nodes, fever, night sweats, fatigue, unintentional weight loss, and symptoms related to the underlying chronic inflammatory condition, such as joint pain or gastrointestinal symptoms
  • Diagnosing the condition typically involves a combination of imaging studies, such as CT or PET scans, a biopsy of affected lymph nodes or tissues, and laboratory tests to analyze blood cell counts and assess markers of inflammation. Molecular testing may also be performed to identify specific genetic abnormalities associated with diffuse large B-cell lymphoma
  • The complications of Diffuse Large B-Cell Lymphoma-Associated with Chronic Inflammation may include lymphoma progression, the spread of cancer to other organs or tissues (metastasis), and complications related to the underlying chronic inflammatory condition, such as organ damage or dysfunction
  • The treatment of Diffuse Large B-Cell Lymphoma-Associated with Chronic Inflammation typically involves chemotherapy, immunotherapy, and/or targeted therapy, depending on the extent of the disease, the presence of specific genetic abnormalities, and the patient's overall health. In some cases, radiation therapy or stem cell transplantation may be recommended
  • The prevention strategies for DLBCL-Associated with Chronic Inflammation may focus on managing and controlling the underlying chronic inflammatory condition through appropriate medical treatment and lifestyle modifications. Regular medical follow-up and monitoring may also help detect and manage potential complications or disease progression
  • The prognosis of Diffuse Large B-Cell Lymphoma-Associated with Chronic Inflammation varies depending on factors such as the stage of the disease at diagnosis, the response to treatment, the presence of specific genetic abnormalities, and the management of the underlying chronic inflammatory condition. Overall, early diagnosis and prompt initiation of appropriate treatment can improve outcomes and may lead to long-term remission in some cases.

Who gets Diffuse Large B-Cell Lymphoma-Associated with Chronic Inflammation? (Age and Sex Distribution)

  • Diffuse Large B-Cell Lymphoma-Associated with Chronic Inflammation can affect individuals of any age, although it most commonly occurs in adults, with a peak incidence in those over the age of 60
  • In general, diffuse large B-cell lymphoma (DLBCL) is the most common type of non-Hodgkin lymphoma; this chronic inflammation subtype accounts for a small percentage of DLBCL cases
  • While DLBCL can occur in both males and females, some studies have suggested a slight male predominance in incidence. However, age and sex distribution can vary depending on the underlying inflammatory condition or predisposing factors

Certain autoimmune diseases, chronic infections, or inflammatory disorders may increase the risk of developing DLBCL Associated with Chronic Inflammation. For example, conditions such as rheumatoid arthritis, inflammatory bowel disease, chronic viral infections (such as hepatitis C or HIV infection), or autoimmune disorders requiring long-term immunosuppressive therapy can predispose individuals to the development of DLBCL in the setting of chronic inflammation.

What are the Risk Factors for Diffuse Large B-Cell Lymphoma-Associated with Chronic Inflammation? (Predisposing Factors)

Several risk factors increase the likelihood of developing Diffuse Large B-Cell Lymphoma-Associated with Chronic Inflammation (DLBCL-CI). These include:

  • Autoimmune diseases: Conditions such as rheumatoid arthritis, systemic lupus erythematosus (SLE), Sjögren's syndrome, and inflammatory bowel disease (Crohn's disease and ulcerative colitis) are associated with chronic inflammation and an increased risk of DLBCL
  • Chronic infections: Persistent infections with certain viruses, bacteria, or parasites can trigger chronic inflammation and contribute to the development of DLBCL. Examples include Epstein-Barr virus (EBV), hepatitis C virus (HCV), human immunodeficiency virus (HIV), Helicobacter pylori (H. pylori), and Chlamydia psittaci
  • Immunosuppressive therapy: Individuals receiving long-term immunosuppressive therapy, such as transplant recipients or patients with autoimmune diseases requiring immunosuppressive medications, have a heightened risk of DLBCL due to their compromised immune function
  • Age: DLBCL-CI predominantly affects older individuals, with a peak incidence in those over the age of 60. Advanced age is a significant risk factor for the development of lymphoma in general
  • Genetic factors: Inherited genetic predispositions may play a role in certain cases of DLBCL. However, the specific genes involved and their contribution to the development of DLBCL associated with chronic inflammation are not yet fully understood
  • Environmental exposures: Exposure to certain environmental factors, such as chemicals, pollutants, or occupational hazards, may increase the risk of DLBCL. However, the relationship between environmental exposures and DLBCL associated with chronic inflammation requires further elucidation

Overall, DLBCL-Associated with Chronic Inflammation is a complex disease influenced by a combination of genetic, environmental, and immunological factors. Identifying and understanding these predisposing factors is crucial for improving risk stratification, early detection, and targeted prevention strategies for high-risk individuals.

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one's chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Diffuse Large B-Cell Lymphoma-Associated with Chronic Inflammation? (Etiology)

The causes of Diffuse Large B-Cell Lymphoma-Associated with Chronic Inflammation (DLBCL-CI) are multifactorial and involve complex interactions between genetic, immunological, environmental, and infectious factors. While the exact etiology remains incompletely understood, several underlying mechanisms have been proposed to contribute to the development of DLBCL in the context of chronic inflammation.

  • Chronic inflammatory microenvironment: Persistent inflammation, characteristic of autoimmune diseases, chronic infections, or inflammatory disorders, creates a pro-inflammatory microenvironment that promotes cell proliferation, genomic instability, and immune dysregulation. Chronic stimulation of the immune system can lead to the activation of oncogenic pathways and the development of lymphoma
  • Immunosuppression: Conditions or treatments that impair immune function, such as immunosuppressive therapy, HIV infection, or organ transplantation, increase the risk of DLBCL by weakening the body's ability to detect and eliminate abnormal cells. Immunosuppression allows for the unchecked proliferation of B-cells, including those with malignant potential, contributing to lymphoma development
  • Viral infections: Certain viruses, such as Epstein-Barr virus (EBV), are associated with an increased risk of DLBCL. EBV, in particular, can infect B-cells and promote their transformation into lymphoma cells through various mechanisms, including viral gene expression, immune evasion, and modulation of cellular signaling pathways
  • Genetic alterations: Genetic abnormalities, including chromosomal translocations, gene mutations, and alterations in signaling pathways, play a critical role in the pathogenesis of DLBCL. These genetic alterations can disrupt cellular processes involved in cell growth, differentiation, and apoptosis, leading to uncontrolled proliferation and lymphomagenesis
  • Microenvironmental interactions: Interactions between lymphoma cells and the surrounding microenvironment, including stromal cells, immune cells, and cytokines, influence tumor behavior and progression. Inflammatory cytokines and chemokines produced within the tumor microenvironment contribute to tumor growth, angiogenesis, and immune evasion, facilitating lymphoma development and dissemination
  • Host factors: Individual genetic susceptibility, immune competence, and host-microbiota interactions may also influence the risk. Due to chronic inflammatory stimuli, genetic variations affecting immune function or inflammatory pathways may predispose certain individuals to lymphoma development

DLBCL-Associated with Chronic Inflammation arises from a complex interplay of genetic, immunological, environmental, and infectious factors. Understanding the underlying etiology and molecular mechanisms driving lymphoma development is essential for developing targeted therapies and preventive strategies to reduce the incidence and improve the outcomes of this aggressive malignancy.

What are the Signs and Symptoms of Diffuse Large B-Cell Lymphoma-Associated with Chronic Inflammation?

The signs and symptoms of Diffuse Large B-Cell Lymphoma-Associated with Chronic Inflammation (DLBCL-CI) can vary depending on the location and extent of the disease, as well as individual patient factors. However, some common signs and symptoms may include:

  • One of the hallmark features of diffuse large B-cell lymphoma (DLBCL) is the presence of painless, swollen lymph nodes, which may be located in the neck, armpits, groin, or other body regions. These lymph nodes may increase in size over time and may be accompanied by tenderness or discomfort
  • B symptoms, including fever, night sweats, and unexplained weight loss, are systemic symptoms often associated with aggressive lymphomas like DLBCL. Fever may be persistent or intermittent, while night sweats may be severe enough to disrupt sleep. Unexplained weight loss, typically defined as losing more than 10% of body weight over six months, may occur despite a normal appetite
  • Many individuals experience generalized fatigue, weakness, and malaise, significantly impacting daily activities and quality of life. Fatigue may be unrelated to physical activity and may not improve with rest
  • Depending on the location of the lymphoma, individuals may experience pain, discomfort, or swelling in affected areas. This may include chest pain, abdominal pain, bone pain, or neurological symptoms if the lymphoma involves the central nervous system
  • In cases where DLBCL affects nearby structures or organs, individuals may experience symptoms related to compression or obstruction. For example, lymphomas in the abdomen may cause abdominal fullness, bloating, constipation, or urinary symptoms due to pressure on the intestines or urinary tract
  • Additional constitutional symptoms may include loss of appetite, nausea, vomiting, itching (pruritus), and recurrent infections. These symptoms may reflect the systemic impact of the lymphoma on the body's immune function and overall health

It is important to note that not all individuals with DLBCL-Associated with Chronic Inflammation will experience the same signs and symptoms, and some individuals may remain asymptomatic until the disease is more advanced. Furthermore, the presence and severity of symptoms can vary widely from person to person. If you experience persistent or concerning symptoms, it is essential to consult with a healthcare provider for further evaluation and diagnosis.

How is Diffuse Large B-Cell Lymphoma-Associated with Chronic Inflammation Diagnosed?

Diagnosing Diffuse Large B-Cell Lymphoma-Associated with Chronic Inflammation typically involves a combination of clinical evaluation, imaging studies, laboratory tests, and tissue biopsy. The diagnostic process aims to confirm the presence of lymphoma, characterize its subtype, assess the extent of disease (staging), and identify any underlying inflammatory conditions or predisposing factors. The diagnostic approaches include:

  • Clinical evaluation: The diagnostic process often begins with a thorough medical history and physical examination. Healthcare providers will inquire about symptoms, medical history, risk factors, and any relevant family history of cancer or autoimmune diseases. Physical examination may include palpation of lymph nodes, assessment of organomegaly (enlarged organs), and evaluation for systemic symptoms such as fever or weight loss
  • Imaging studies: Imaging studies such as computed tomography (CT) scans, positron emission tomography (PET) scans, magnetic resonance imaging (MRI), or ultrasound may be performed to assess the extent of disease involvement and detect abnormalities such as enlarged lymph nodes, organ enlargement, or masses
  • Laboratory tests: Blood tests may be ordered to evaluate blood cell counts, assess liver and kidney function, and detect inflammation or immune dysregulation markers. Specific tests may also be performed to identify infectious agents such as Epstein-Barr virus (EBV) or hepatitis C virus (HCV) if suspected
  • Tissue biopsy: The definitive diagnosis of diffuse large B-cell lymphoma (DLBCL) requires the examination of tissue samples obtained through biopsy. This may involve the removal of a lymph node (lymph node biopsy), a bone marrow (bone marrow biopsy) sample, or a biopsy of other affected tissues or organs. The biopsy sample is examined by a pathologist who evaluates the cellular morphology, immunophenotype, and genetic features to confirm the diagnosis of DLBCL and characterize its subtype
  • Immunohistochemistry and molecular testing: Immunohistochemical analysis of biopsy samples helps to determine the expression of specific proteins characteristic of DLBCL and identify markers associated with chronic inflammation or specific lymphoma subtypes. Molecular testing, such as fluorescence in situ hybridization (FISH) or polymerase chain reaction (PCR), may be performed to detect genetic abnormalities or infectious agents within the lymphoma cells
  • Staging: Once a DLBCL diagnosis is confirmed, staging tests may be conducted to determine the extent of disease spread and guide treatment decisions. Staging typically involves additional imaging studies, such as CT or PET scans, bone marrow biopsy, and lumbar puncture (if central nervous system involvement is suspected)

The diagnostic process for DLBCL-Associated with Chronic Inflammation requires a multidisciplinary approach involving oncologists, hematopathologists, radiologists, and other healthcare professionals. Accurate diagnosis and staging are essential for developing an appropriate treatment plan and optimizing patient outcomes.

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Diffuse Large B-Cell Lymphoma-Associated with Chronic Inflammation?

Diffuse Large B-Cell Lymphoma-Associated with Chronic Inflammation (DLBCL-CI) can lead to several potential complications, which may arise from the disease, its treatment, or related underlying conditions. These complications can significantly impact a patient's quality of life and overall prognosis. Some of these include:

  • Disease progression: Diffuse large B-cell lymphoma (DLBCL) is an aggressive lymphoma that can rapidly progress if left untreated or inadequately controlled. Untreated lymphoma can infiltrate and damage nearby organs or tissues, leading to organ dysfunction and potentially life-threatening complications
  • Treatment-related side effects: Chemotherapy, radiation therapy, and other treatments used to manage DLBCL can cause a range of side effects, including nausea, vomiting, fatigue, hair loss, anemia, neutropenia (low white blood cell count), thrombocytopenia (low platelet count), and increased susceptibility to infections. These side effects can affect a patient's physical and emotional well-being and may require supportive care or dose modifications
  • Immunosuppression: Certain treatments for DLBCL, such as chemotherapy and immunotherapy, can suppress the immune system, increasing the risk of infections. Patients with DLBCL associated with chronic inflammation may already have compromised immune function due to underlying conditions or immunosuppressive medications, further heightening the risk of infection-related complications
  • Central nervous system (CNS) involvement: DLBCL can involve the central nervous system, leading to neurological symptoms such as headaches, seizures, cognitive impairment, or motor deficits. CNS involvement may occur at the time of diagnosis or as a relapse following treatment and requires prompt evaluation and management
  • Secondary cancers: Some treatments, particularly radiation therapy and certain chemotherapy regimens, can increase the risk of developing secondary cancers later in life. Long-term surveillance and monitoring for secondary malignancies are essential for patients treated for DLBCL
  • Psychosocial impact: Coping with a diagnosis of DLBCL and undergoing treatment can have a significant psychosocial impact on patients and their families. Anxiety, depression, fear of recurrence, financial strain, and changes in body image are common emotional and psychological challenges faced by the affected individuals.
  • Complications from underlying inflammatory conditions: DLBCL-CI often occurs in the context of underlying autoimmune diseases, chronic infections, or inflammatory disorders. Complications related to these underlying conditions, such as disease flares, organ damage, or treatment-related toxicities, may exacerbate the clinical course of DLBCL and complicate its management

Managing and mitigating these complications requires a comprehensive and multidisciplinary approach, with close monitoring, supportive care, and tailored interventions to address individual patient needs and optimize treatment outcomes. Early recognition and prompt management of complications are essential for improving patient quality of life and overall prognosis.

How is Diffuse Large B-Cell Lymphoma-Associated with Chronic Inflammation Treated?

The treatment approach for Diffuse Large B-Cell Lymphoma-Associated with Chronic Inflammation (DLBCL-CI) typically involves a combination of chemotherapy, immunotherapy, targeted therapy, radiation therapy, and supportive care. The specific treatment regimen selected for each patient depends on factors such as the stage of the disease, the patient's overall health and preferences, the type and severity of the underlying inflammatory condition, and the molecular characteristics of the lymphoma. The main treatment modalities include:

  • Chemotherapy: The cornerstone of treatment for diffuse large B-cell lymphoma (DLBCL) is chemotherapy, which typically involves combination chemotherapy regimens such as R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) or similar regimens. Chemotherapy is administered in cycles over several weeks, with the goal of killing cancer cells and inducing remission
  • Immunotherapy: Monoclonal antibody therapy, particularly rituximab, is often combined with chemotherapy to treat DLBCL. Rituximab targets and destroys CD20-positive B-cells, including malignant lymphoma cells, enhancing the effectiveness of chemotherapy and improving treatment outcomes
  • Targeted therapy: In cases of DLBCL with specific molecular abnormalities or genetic alterations, targeted therapy agents may be used to inhibit specific signaling pathways involved in lymphoma growth and survival
  • Radiation therapy: Radiation therapy may be used as part of the treatment approach for localized DLBCL or as consolidation therapy following chemotherapy to target residual disease. Radiation therapy delivers high-energy beams to specific areas of the body affected by lymphoma, helping to destroy cancer cells and reduce the risk of recurrence
  • Stem cell transplantation: For patients with relapsed/refractory DLBCL or high-risk disease, high-dose chemotherapy followed by autologous stem cell transplantation (ASCT) may be considered. ASCT involves the infusion of previously collected stem cells to restore bone marrow function following high-dose chemotherapy
  • Supportive care measures are essential components of DLBCL treatment and aim to manage treatment-related side effects, optimize patient comfort, and improve overall quality of life. Supportive care interventions may include medications to control nausea, pain, or infection, blood transfusions to address anemia or thrombocytopenia, and psychological support for coping with the emotional impact of the disease

Eligible patients with DLBCL-CI may be considered for participation in clinical trials evaluating novel treatment approaches, including new chemotherapy regimens, immunotherapy agents, targeted therapies, or combination strategies

DLBCL-Associated with Chronic Inflammation treatment requires a personalized approach tailored to individual patient characteristics and disease biology. Close collaboration between oncologists, hematologists, radiation oncologists, pathologists, and other healthcare professionals is essential for developing and implementing an optimal treatment plan that maximizes treatment efficacy while minimizing treatment-related toxicity and complications. Monitoring and follow-up are important to assess treatment response, manage side effects, and address any disease recurrence or progression.

What is the Prognosis of Diffuse Large B-Cell Lymphoma-Associated with Chronic Inflammation? (Outcomes/Resolutions)

The prognosis of Diffuse Large B-Cell Lymphoma-Associated with Chronic Inflammation (DLBCL-CI) varies depending on factors such as the stage of the disease, the patient's overall health and fitness, underlying inflammatory conditions, molecular characteristics of the lymphoma, and the response to treatment. While diffuse large B-cell lymphoma (DLBCL) is considered an aggressive form of non-Hodgkin lymphoma, advancements in treatment approaches have improved overall outcomes in recent years.

Several prognostic factors have been identified that can influence the outcomes of DLBCL, including the International Prognostic Index (IPI), which incorporates factors such as age, disease stage, lactate dehydrogenase (LDH) levels, performance status, and extranodal involvement. Additional molecular and genetic markers, such as the presence of specific genetic abnormalities or gene expression profiles, may also provide prognostic information.

  • The majority of patients will achieve a complete or partial response to initial treatment, particularly with combination chemotherapy regimens such as R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone). The extent and duration of treatment response are important predictors of long-term outcomes
  • Following initial treatment, some patients with DLBCL-CI will achieve remission with no evidence of active disease. However, DLBCL tends to recur, and relapses can occur months or years after initial treatment. The likelihood of relapse depends on various factors, including disease stage, molecular subtype, and response to treatment
  • The overall survival (OS) and progression-free survival (PFS) rates for DLBCL Associated with Chronic Inflammation have improved with the introduction of rituximab and other targeted therapies. The five-year survival rate for DLBCL ranges from approximately 60% to 70%, with higher survival rates observed in patients who achieve a complete response to initial treatment
  • DLBCL-CI and its treatment can be associated with various complications, including treatment-related toxicities, infections, secondary malignancies, and relapse. Close monitoring and supportive care measures are essential for managing these complications and optimizing patient outcomes

The prognosis of Diffuse Large B-Cell Lymphoma-Associated with Chronic Inflammation is highly individualized, and outcomes can vary widely from one patient to another. Factors such as response to treatment, disease aggressiveness, and the presence of comorbidities will influence the overall prognosis and long-term survival.

Although the condition remains challenging to treat, advances in treatment strategies, including immunotherapy, targeted therapy, and stem cell transplantation, have contributed to improved outcomes and survival rates for many patients. Early diagnosis, risk stratification, and personalized treatment approaches are crucial for optimizing prognosis and achieving favorable long-term outcomes in individuals with DLBCL-Associated with Chronic Inflammation.

Additional and Relevant Useful Information for Diffuse Large B-Cell Lymphoma-Associated with Chronic Inflammation:

Diffuse Large B-Cell Lymphoma-Associated with Chronic Inflammation is often diagnosed at an advanced stage due to its indolent initial presentation and association with chronic conditions. Treatment typically involves a combination of chemotherapy and targeted therapies, similar to other forms of diffuse large B-cell lymphoma (DLBCL), but the response to treatment can be variable due to the underlying inflammatory condition. Prognosis can be poorer compared to other DLBCL subtypes, underscoring the importance of early detection and treatment of the associated chronic inflammatory condition to mitigate the risk of lymphoma development potentially.

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Krish Tangella MD, MBA picture
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Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team
Sumanth Marupuru picture
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Sumanth Marupuru

Editorial Staff

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