Deepika vegiraju at 02:26, 28 June 2020

2020-06-28T02:26:13Z

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Diffuse large B-cell lymphoma (DLBCL), a form of [[non-Hodgkin lymphoma]], is the most common blood cancer. [[Lymphomas]] occur when cells of the immune system, known as [[B lymphocytes]], grow and multiply uncontrollably.
DLBCL occurs mostly in adults and is a fast-growing (aggressive) lymphoma. It can start in the [[lymph nodes]] or outside of the lymphatic system in the [[gastrointestinal tract]], [[testes]], [[thyroid]], skin, breast, bone, or brain.

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=='''Signs and symptoms'''==
the first sign of DLBCL is a painless rapid swelling in the neck, armpit, abdomen, or groin caused by enlarged lymph nodes. For some people, the swelling may be painful. Other symptoms include night sweats, unexplained fevers, and weight loss.

=='''cause'''==
The causes of diffuse large B-cell lymphoma are not well understood. Usually DLBCL arises from normal B cells, but it can also represent a [[malignant transformation]] of other types of lymphoma (particularly [[marginal zone lymphoma]]s or, in rare cases termed [[Richter's transformation]], [[chronic lymphocytic leukemia]].
An underlying [[immunodeficiency]] is a significant risk factor for development of the disease.
Infections with the [[Epstein–Barr virus]],[[Kaposi's sarcoma-associated herpesvirus]],
[[human immunodeficiency virus]] (i.e. HIV),and the ''[[Helicobacter pylori]]'' bacterium are also associated with the development of certain subtypes of diffuse large B-cell lymphoma.
However, most cases of this disease are associated with the unexplained step-wise acquisition of increasing numbers of [[gene mutations]] and changes in gene expression that occur in, and progressively promote the malignant behavior of, certain B-cell types.

=='''Diagnosis'''==
Diagnosis of DLBCL is made by removing a portion of the tumor through a [[biopsy]], and then examining this tissue using a microscope. Usually a [[Hematopathology|hematopathologist]] makes this diagnosis.
Numerous subtypes of DLBCL have been identified which differ in their clinical presentations, biopsy findings, aggressive characteristics, [[Prognosis|prognoses]], and recommended treatments

Microscopic examinations of involved tissues reveal large neoplastic cells that are typically classified as B-cells based on their expression of B-cell marker proteins (e.g. [[CD20]], [[CD19]], [[CD22]], [[CD79]], PAX5, [[POU2AF1|BOB1]], [[SLC22A2|OCT2]], an [[immunoglobulin]] [usually [[IgM]] but occasionally [[IgG]] or [[IgA]])] [[CD30]]and in ~20-25% of cases [[PD-L1]] or [[PD-L2]] (PD-L1 and PD-L2 are [[transmembrane protein]]s that normally function to suppress attack by the immune system). These cells arrange in a diffuse pattern, efface the tissues' architecture, and resemble [[Centroblast]] cells (80% of cases), [[Immunoblast]] cells (8-10% of cases), or [[Anaplasia|anaplastic]] cells (9% of cases; anaplastic cells have bizarre nuclei and other features that may mimic the [[Reed-Sternberg cells]] of [[Hodgkin disease]] or the neoplastic cells of [[anaplastic large cell lymphoma]]).

Rarely, these neoplastic cells are characterized as having signet ring or spindle shaped nuclei, prominent [[cytoplasm]]ic granules, multiple [[microvillus]] projections, or, when viewed by [[electron microscopy]]

=='''Genetics'''==
Most cases of DLBCL, NOS appear to result at least in part from the step-wise development of [[gene]] changes such as [[mutations]], [[Regulation of transcription in cancer|altered expressions]], [[Gene amplification|amplifications]] (i.e. increases in the number of copies of specific genes), and [[Chromosomal translocation|tranlocations]] from normal sites to other chromosomal sites.

Changes in the following genes occur frequently in, and are suspected of contributing to, this disease's development and/or progression.

[[Bcl-2|BCL2]],[[BCL6]],[[MYC]],[[EZH2]],[[MYD88]] ,[[CREBBP]],[[CD79A]],[[PAX5]]

=='''Treatment '''==
Treatment may differ depending on the location of the [[tumor]] and the subtype of [[lymphoma]].
For those who have advanced DCBCL and have not been treated previously, a combination of [[chemotherapy]] and the [[monoclonal antibody]] [[rituximab]] (Rituxan) (R-CHOP) may be tried.
[[Axicabtagene ciloleucel]] (brand name: Yescarta), a type of [[gene therapy]], has been approved by the United States FDA to treat DCBCL that is not responding to at least two treatment attempts or has returned after being treated before.
[[A stem cell transplant]] may also be an option if DLBCL returns or relapses.

The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition.

*'''[[Tisagenlecleucel-T]]''' (Brand name: Kymriah)KYMRIAH is a CD19-directed genetically modified autologous T-cell [[immunotherapy]] indicated for the treatment of patients with diffuse large B-cell lymphoma, high-grade B-cell lymphoma, or DLBCL arising from follicular lymphoma who received two or more lines of systemic therapy.

*'''[[Rituximab and hyaluronidase]]''' human (Brand name: Rituxan Hycela)Treatment of adult patients with previously untreated diffuse large B-cell lymphoma in combination with [[cyclophosphamide]], [[doxorubicin]], [[vincristine]], [[prednisone]] (CHOP) or other [[anthracycline]]-based chemotherapy regimens.
*'''[[Axicabtagene ciloleucel]]''' (Brand name: Yescarta)Treatment of adult patients with relapsed or refractory large B-cell lymphoma after two or more lines of systemic therapy, including diffuse large B-cell lymphoma (DLBCL) not otherwise specified, primary mediastinal large B-cell lymphoma, high-grade B-cell lymphoma, and DLBCL arising from [[follicular lymphoma]].
{{Hematological malignancy histology}}
{{Chromosomal abnormalities}}

[[Category:Lymphoma]]
[[Category:Non-Hodgkin lymphoma]]
[[Category:Epstein–Barr virus-associated diseases]]
{{rarediseases}}
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Diffuse Large B-Cell Lymphoma - Revision history

Deepika vegiraju at 02:26, 28 June 2020