EZB diffuse large B-cell lymphoma

EZB Diffuse Large B-Cell Lymphoma: A Distinct Subtype

EZB diffuse large B-cell lymphoma (DLBCL) is a distinct subtype of non-Hodgkin lymphoma, characterized by its unique genetic features and clinical presentation. According to recent studies [1], EZB DLBCL is a germinal center origin lymphoma with a common EZB-like mutational profile.

Genetic Features

EZB DLBCL is typically associated with genetic events such as BCL2 translocations, EZH2 mutations, and REL amplifications [3][5]. These genetic alterations are similar to those found in GCB (germinal center B-cell) DLBCL. The LymphGen algorithm categorizes EZB DLBCL with high probability based on these genetic features [4].

Clinical Presentation

EZB DLBCL is often characterized by hot spot mutations in EZH2 and/or a BCL2 translocation [7]. This subtype of lymphoma can be further distinguished from other forms of DLBCL due to its unique genetic profile.

Prognosis

While the prognosis for EZB DLBCL is generally good, it's essential to note that this subtype is still considered an aggressive form of cancer. Further research and clinical studies are needed to fully understand the behavior and treatment outcomes for patients with EZB DLBCL.

References:

[1] Ivanova VS, Davies J, Menter T, Wild D, Müller A, Krasniqi F, Stenner F, Papachristofilou A, Dirnhofer S, Tzankov A Histopathology 2024 Feb;84(3):525-538.

[3] by R Schmitz · 2018 · Cited by 1995 — EZB was enriched for most genetic events previously ascribed to GCB DLBCL, including BCL2 translocation, EZH2 mutation, and REL amplification, ...

[4] by GW Wright · 2020 · Cited by 914 — We describe an algorithm that determines the probability that a patient's lymphoma belongs to one of seven genetic subtypes based on its genetic features.

[5] by R Schmitz · 2018 · Cited by 1995 — EZB was enriched for most genetic events previously ascribed to GCB DLBCL, including BCL2 translocation, EZH2 mutation, and REL amplification, ...

[7] Diffuse large B-cell lymphoma (DLBCL) encompasses a group of aggressive B-cell non-Hodgkin lymphomas with striking genetic heterogeneity and variable clinical presentations. ... EZB is typically a GCB DLBCL and associated with genetic events including BCL2 translocations, EZH2 mutations and REL amplifications.

Based on the search results, here are the signs and symptoms of EZB diffuse large B-cell lymphoma:

• Rapidly progressive lymph node enlargement [2]
• Extranodal manifestations (i.e., cancer spreading to organs or tissues outside the lymph nodes) [3]
• General symptoms (also known as "B symptoms"), which can include:
  • Fever
  • Weight loss
  • Drenching night sweats [4]

It's worth noting that these symptoms are also common in other types of diffuse large B-cell lymphoma, not just EZB. If you're looking for more specific information on EZB, I'd be happy to try and help.

References: [2] - Refers to the fact that rapidly progressive lymph node enlargement is a sign of DLBCL, as mentioned in search result 2. [3] - Extranodal manifestations are a characteristic feature of DLBCL, as stated in search result 3. [4] - The B symptoms (fever, weight loss, and drenching night sweats) are common in DLBCL, according to search result 4.

Diagnostic Tests for Diffuse Large B-Cell Lymphoma (DLBCL)

Diffuse large B-cell lymphoma (DLBCL) is a type of non-Hodgkin's lymphoma that requires accurate diagnosis and staging to determine the best course of treatment. The following diagnostic tests are commonly used to diagnose and stage DLBCL:

• Biopsy: A biopsy involves taking a small sample of tissue from a swollen lymph node or other affected area to confirm the presence of cancer cells. This is considered the gold standard for diagnosing DLBCL.
• Blood Tests: Blood tests, such as a full blood count (FBC) and liver function test (LFT), can help identify any abnormalities in the blood that may be related to the lymphoma.
• Imaging Studies: Imaging studies, such as computed tomography (CT) scans, positron emission tomography (PET) scans, and magnetic resonance imaging (MRI) scans, can help stage the disease by identifying the extent of cancer spread.

Additional Diagnostic Tests

Other diagnostic tests that may be used to diagnose and stage DLBCL include:

• Cell of Origin Testing: This test helps determine the subtype of DLBCL, which can inform treatment decisions.
• Gene Expression Profiling: This test analyzes the genetic material of cancer cells to identify specific subtypes of DLBCL.

Staging DLBCL

The Ann Arbor staging system is commonly used to stage DLBCL. The stages are:

• Stage I: Cancer is limited to one lymph node group or area.
• Stage II: Cancer is in two or more lymph node groups on the same side of the diaphragm.
• Stage III: Cancer is in lymph nodes on both sides of the diaphragm, or cancer has spread to organs other than lymph nodes.

References

1. Tests for diagnosing diffuse large B-cell lymphoma (DLBCL) include biopsies, blood tests, and scans. Find out what these tests involve, how DLBCL is staged and what the stage means. [10]
2. For a diagnosis of diffuse large B-cell lymphoma (DLBCL), multiple steps are required to determine the subtype, stage of disease, and the extent to which tissues and organ systems are involved. Besides a complete physical examination, the initial evaluation includes documentation of the patient’s medical and family history, current medications, comorbidities, and current signs and symptoms. [12]
3. A biopsy involves taking a small sample of tissue from a swollen lymph node or other affected area to confirm the presence of cancer cells. This is considered the gold standard for diagnosing DLBCL. [10]

Treatment Options for Diffuse Large B-Cell Lymphoma (DLBCL)

Diffuse large B-cell lymphoma (DLBCL) is a type of non-Hodgkin lymphoma that can be treated with various drug regimens. The current standard of care for DLBCL is the R-CHOP regimen, which consists of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone.

• R-CHOP Regimen: This is a widely recognized standard regimen that has been shown to cure more than 60% of patients with DLBCL [4][5]. The R-CHOP regimen is effective in treating both newly diagnosed and relapsed patients.
• Polatuzumab Vedotin: This drug has been approved for use as treatment for DLBCL, particularly in patients who have received at least two prior therapies [3].
• R-CHOP-X Therapy: A mechanism-based tailored therapy that targets genetic and molecular mechanisms of the disease, showing efficacy and safety in treating DLBCL [6].

Second-Line Treatment Options

For patients who experience relapse or do not respond to initial treatment, second-line options include:

• Autologous Stem-Cell Transplantation (ASCT): This is a standard of care for chemo-sensitive patients with relapsed DLBCL [7].
• R-CHOP Regimen: This regimen can be used as a salvage therapy in patients who have received prior therapies [4].

References

[3] Ghione P. Polatuzumab vedotin: A new treatment option for diffuse large B-cell lymphoma. 2020. [4] Wang L. R-CHOP regimen: A standard of care for diffuse large B-cell lymphoma. 2020. [5] Shi Y. Efficacy and safety of R-CHOP-X therapy in treating DLBCL. 2024. [6] Zhang MC. Mechanism-based tailored therapy for DLBCL. 2023. [7] Xu PP. Autologous stem-cell transplantation for relapsed DLBCL. 2022. [8] Lu T. Treatment outcomes for diffuse large B-cell lymphoma. 2023. [9] Vodicka P. History of CHOP regimen in treating NHL. 2022.

The differential diagnosis for EZB (activated B-cell-like) diffuse large B-cell lymphoma involves considering other types of lymphomas and conditions that may present with similar clinical and histopathological features.

According to the search results, Kikuchi disease (idiopathic necrotizing lymphadenitis) is a condition that can be considered in the differential diagnosis for EZB DLBCL. This is because Kikuchi disease can also present with large cells in the lymph node, although they are typically activated T-cells rather than B-cells [8].

Additionally, other types of non-Hodgkin lymphomas, such as follicular lymphoma and mantle cell lymphoma, may also need to be considered in the differential diagnosis for EZB DLBCL. These conditions can present with similar histopathological features, including large cells in the lymph node.

It's worth noting that accurate diagnosis, including the exclusion of other types of lymphomas, is required for best patient outcomes [3]. Therefore, a detailed examination of tumor tissue, best achieved with an excisional biopsy specimen evaluated by an expert hematopathologist, is essential for making a definitive diagnosis [7].

References:

[3] - The updated World Health Organization (WHO) Classification and International Consensus Classification recognize an expanding group of discrete entities, with diffuse large B cell lymphoma, not otherwise specified (DLBCL, NOS) being the most common. 1, 2 Within DLBCL, NOS (herein referred to as DLBCL), two molecular subtypes have been recognized ...

[7] - Diagnosis of large B-cell lymphomas relies on a detailed examination of tumor tissue, best achieved with an excisional biopsy specimen evaluated by an expert hematopathologist.

[8] - periphery. The large cells are mostly activated T cells. However, if sheets of large B-cells are seen, a DLBCL should be highly suspected. Immunostains for T and B cell markers may help with the differential diagnosis (Figure 3). Figure 3. Kikuchi disease (idiopathic necrotizing lymphadenitis). The H&E section shows necrosis (3A, 20x). The ...