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__NOTOC__
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{{Diffuse large B cell lymphoma}}
{{Diffuse large B cell lymphoma}}
{{CMG}}; {{AE}} {{AS}}
{{CMG}}; {{AE}} {{AS}} {{AHS}}
==Overview==
==Overview==
'''Diffuse large B-cell lymphoma (DLBCL or DLBL)''' is a [[cancer]] of [[B cell]]s, a type of [[white blood cell]] responsible for producing [[antibody|antibodies]]. Diffuse large B cell lymphoma may be classified based on location into nodal and extranodal disease and based on [[molecular]], [[genetic]], and [[immunohistochemical]] features into more than 20 subgroups. Diffuse large B cell lymphoma may be classified into 2 subtypes based on [[gene expression]] profiles. The progression to diffuse large B cell lymphoma involves the [[microRNAs]] (miRNAs). On microscopic histopathological analysis, diffuse large B cell lymphoma can be divided into three variants: centroblastic, immunoblastic, and anaplastic. There are no established causes for diffuse large B cell lymphoma. Diffuse large B cell lymphoma is the most common type of [[non-Hodgkin lymphoma]] among adults,<ref name="Blood1997">{{cite journal |pmid=9166827 |year=1997 |title=A clinical evaluation of the International Lymphoma Study Group classification of non-Hodgkin's lymphoma. The Non-Hodgkin's Lymphoma Classification Project |journal=Blood |volume=89 |issue=11 |pages=3909–18 |url=http://www.bloodjournal.org/content/89/11/3909 }}</ref> with an annual [[Incidence (epidemiology)|incidence]] of 7–8 cases per 100,000 people per year.<ref name="Morton2006">{{cite journal |doi=10.1182/blood-2005-06-2508 |pmid=16150940 |pmc=1895348 |title=Lymphoma incidence patterns by WHO subtype in the United States, 1992-2001 |journal=Blood |volume=107 |issue=1 |pages=265–76 |year=2006 |last1=Morton |first1=L. M. |last2=Wang |first2=S. S. |last3=Devesa |first3=S. S. |last4=Hartge |first4=P |last5=Weisenburger |first5=D. D. |last6=Linet |first6=M. S. }}</ref><ref name="Smith2011">{{cite journal |doi=10.1038/bjc.2011.450 |pmid=22045184 |pmc=3242607 |title=Incidence of haematological malignancy by sub-type: A report from the Haematological Malignancy Research Network |journal=British Journal of Cancer |volume=105 |issue=11 |pages=1684–92 |year=2011 |last1=Smith |first1=A |last2=Howell |first2=D |last3=Patmore |first3=R |last4=Jack |first4=A |last5=Roman |first5=E }}</ref> This cancer occurs primarily in older individuals, with a median age of diagnosis at approximately 70 years of age,<ref name="Smith2011" /> though it can also occur in children and young adults in rare cases.<ref name="Smith2010">{{cite journal |doi=10.1111/j.1365-2141.2009.08010.x |pmid=19958356 |pmc=3066245 |title=The Haematological Malignancy Research Network (HMRN): A new information strategy for population based epidemiology and health service research |journal=British Journal of Haematology |volume=148 |issue=5 |pages=739–53 |year=2010 |last1=Smith |first1=Alexandra |last2=Roman |first2=Eve |last3=Howell |first3=Debra |last4=Jones |first4=Richard |last5=Patmore |first5=Russell |last6=Jack |first6=Andrew |author7=Haematological Malignancy Research Network }}</ref> The most potent risk factor in the development of diffuse large B cell lymphoma is [[immunodeficiency]]. Diffuse large B cell lymphoma must be differentiated from other diseases such as [[follicular lymphoma]], [[Mucosa-Associated Lymphatic Tissue lymphoma]](MALT), [[small cell lymphocytic lymphoma]], and [[Mantle cell lymphoma]] (MCL). The prognosis of diffuse large B cell lymphoma depends on the stage of the disease. Diffuse large B cell lymphoma is associated with a 5 year survival rate ranging from 70% to more than 90% among children. According to the Lugano classification, there are four stages of diffuse large B cell lymphoma based on the number of nodes involved and extranodal status. The most common symptoms of diffuse large B cell lymphoma include a rapidly enlarging, non-painful mass that may be located in neck, groin or abdomen. The less common symptoms of diffuse large B cell lymphoma include fever, weight loss and drenching night sweats. Common physical examination findings of diffuse large B cell lymphoma include a non tender mass which can arise in virtually any part of the body. Laboratory tests of diffuse large B cell lymphoma include [[complete blood count]], blood chemistry studies, [[flow cytometry]], [[immunohistochemistry]], [[cytology]], [[genetic testing]], and [[immunophenotyping]]. [[CT]] scan, [[MRI]], and [[PET]] may be helpful in the diagnosis of diffuse large B cell lymphoma. Lymph node or extranodal tissue [[biopsy]] is diagnostic of large B cell lymphoma. Other diagnostic studies for diffuse large B cell lymphoma include [[laparoscopy]], [[laparotomy]], and [[bone marrow aspiration]] and biopsy. The optimal therapy for diffuse large B cell lymphoma depends on the stage at diagnosis. The predominant therapy for diffuse large B cell lymphoma is [[chemotherapy]]. Adjunctive [[radiotherapy]] may be required. Active areas of research include genetic studies to assess the genetic role in diffuse large B cell lymphoma and novel therapeutic regimens that evaluate the efficacy and safety of novel regimens in in newly diagnosed diffuse large B cell lymphoma.
'''Diffuse large B-cell lymphoma (DLBCL or DLBL)''' is a [[cancer]] of [[B cell]]s, a type of [[white blood cell]] responsible for producing [[antibody|antibodies]]. Diffuse large B cell lymphoma may be classified based on location into nodal and extranodal disease and based on [[molecular]], [[genetic]], and [[immunohistochemical]] features into more than 20 subgroups. Diffuse large B cell lymphoma may be classified into 2 subtypes based on [[gene expression]] profiles. The progression to diffuse large B cell lymphoma involves the [[microRNAs]] (miRNAs). On microscopic histopathological analysis, diffuse large B cell lymphoma can be divided into three variants: centroblastic, immunoblastic, and anaplastic. There are no established causes for diffuse large B cell lymphoma. Diffuse large B cell lymphoma is the most common type of [[non-Hodgkin lymphoma]] among adults, with an annual [[Incidence (epidemiology)|incidence]] of 7–8 cases per 100,000 people per year. This cancer occurs primarily in older individuals, with a median age of diagnosis at approximately 70 years of age, though it can also occur in children and young adults in rare cases. The most potent risk factor in the development of diffuse large B cell lymphoma is [[immunodeficiency]]. Diffuse large B cell lymphoma must be differentiated from other diseases such as [[follicular lymphoma]], [[Mucosa-Associated Lymphatic Tissue lymphoma]](MALT), [[small cell lymphocytic lymphoma]], and [[Mantle cell lymphoma]] (MCL). The prognosis of diffuse large B cell lymphoma depends on the stage of the disease. Diffuse large B cell lymphoma is associated with a 5 year survival rate ranging from 70% to more than 90% among children. According to the Lugano classification, there are four stages of diffuse large B cell lymphoma based on the number of nodes involved and extranodal status. The most common symptoms of diffuse large B cell lymphoma include a rapidly enlarging, non-painful mass that may be located in neck, groin or abdomen. The less common symptoms of diffuse large B cell lymphoma include fever, weight loss and drenching night sweats. Common physical examination findings of diffuse large B cell lymphoma include a non tender mass which can arise in virtually any part of the body. Laboratory tests of diffuse large B cell lymphoma include [[complete blood count]], blood chemistry studies, [[flow cytometry]], [[immunohistochemistry]], [[cytology]], [[genetic testing]], and [[immunophenotyping]]. [[CT]] scan, [[MRI]], and [[PET]] may be helpful in the diagnosis of diffuse large B cell lymphoma. Lymph node or extranodal tissue [[biopsy]] is diagnostic of large B cell lymphoma. Other diagnostic studies for diffuse large B cell lymphoma include [[laparoscopy]], [[laparotomy]], and [[bone marrow aspiration]] and biopsy. The optimal therapy for diffuse large B cell lymphoma depends on the stage at diagnosis. The predominant therapy for diffuse large B cell lymphoma is [[chemotherapy]]. Adjunctive [[radiotherapy]] may be required. Active areas of research include genetic studies to assess the genetic role in diffuse large B cell lymphoma and novel therapeutic regimens that evaluate the efficacy and safety of novel regimens in in newly diagnosed diffuse large B cell lymphoma.


==Classification==
==Classification==
Diffuse large B cell lymphoma may be classified based on location into nodal and extranodal disease and based on [[molecular]], [[genetic]], and [[immunohistochemical]] features into more than 20 subgroups.
Diffuse large B cell lymphoma may be classified based on location into nodal and extranodal disease and based on [[molecular]], [[genetic]], and [[immunohistochemical]] features into more than 20 subgroups.
==Pathophysiology==
==Pathophysiology==
Diffuse large B cell lymphoma may be classified into 2 subtypes based on [[gene expression]] profiles. The progression to diffuse large B cell lymphoma involves the [[microRNAs]] (miRNAs). On microscopic histopathological analysis, diffuse large B cell lymphoma can be divided into three variants: centroblastic, immunoblastic, and anaplastic.
Diffuse large B cell lymphoma is mainly caused by [[genetic mutations]]. Genetic expression of [[germinal centers]] [[B cell]] like are associated with favourable prognosis. Some studies have established an association between [[microRNA]] expression and B cell lymphoma pathogenesis. The studies showed poor prognosis of microRNA expressed lymphomas. [[MicroRNAs]] participate in development of [[B cell receptor]] signalling, B cell migration, and [[Immunoglobulin class switching|class switching of immunoglobulins]]. On microscopic pathology, diffuse large B cell lymphoma has three variant pictures which include [[Centroblastic and centrocytic lymphoma|centroblastic]], [[Immunoblastic Lymphadenopathy|immunoblastic]], and [[anaplastic]] forms of DLBCL.
==Causes==
==Causes==
There are no established causes for diffuse large B cell lymphoma.
There are no established causes for diffuse large B cell lymphoma.
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Diffuse large B cell lymphoma must be differentiated from other diseases such as [[follicular lymphoma]], [[Mucosa-Associated Lymphatic Tissue lymphoma]](MALT), [[small cell lymphocytic lymphoma]], and [[mantle cell lymphoma]] (MCL).
Diffuse large B cell lymphoma must be differentiated from other diseases such as [[follicular lymphoma]], [[Mucosa-Associated Lymphatic Tissue lymphoma]](MALT), [[small cell lymphocytic lymphoma]], and [[mantle cell lymphoma]] (MCL).
==Epidemiology and demographics==
==Epidemiology and demographics==
Diffuse large B cell lymphoma is the most common type of [[non-Hodgkin lymphoma]] among adults,<ref name="Blood1997">{{cite journal |pmid=9166827 |year=1997 |title=A clinical evaluation of the International Lymphoma Study Group classification of non-Hodgkin's lymphoma. The Non-Hodgkin's Lymphoma Classification Project |journal=Blood |volume=89 |issue=11 |pages=3909–18 |url=http://www.bloodjournal.org/content/89/11/3909 }}</ref> with an annual [[Incidence (epidemiology)|incidence]] of 7–8 cases per 100,000 people per year.<ref name="Morton2006">{{cite journal |doi=10.1182/blood-2005-06-2508 |pmid=16150940 |pmc=1895348 |title=Lymphoma incidence patterns by WHO subtype in the United States, 1992-2001 |journal=Blood |volume=107 |issue=1 |pages=265–76 |year=2006 |last1=Morton |first1=L. M. |last2=Wang |first2=S. S. |last3=Devesa |first3=S. S. |last4=Hartge |first4=P |last5=Weisenburger |first5=D. D. |last6=Linet |first6=M. S. }}</ref><ref name="Smith2011">{{cite journal |doi=10.1038/bjc.2011.450 |pmid=22045184 |pmc=3242607 |title=Incidence of haematological malignancy by sub-type: A report from the Haematological Malignancy Research Network |journal=British Journal of Cancer |volume=105 |issue=11 |pages=1684–92 |year=2011 |last1=Smith |first1=A |last2=Howell |first2=D |last3=Patmore |first3=R |last4=Jack |first4=A |last5=Roman |first5=E }}</ref> This cancer occurs primarily in older individuals, with a median age of diagnosis at approximately 70 years of age,<ref name="Smith2011" /> though it can also occur in children and young adults in rare cases.<ref name="Smith2010">{{cite journal |doi=10.1111/j.1365-2141.2009.08010.x |pmid=19958356 |pmc=3066245 |title=The Haematological Malignancy Research Network (HMRN): A new information strategy for population based epidemiology and health service research |journal=British Journal of Haematology |volume=148 |issue=5 |pages=739–53 |year=2010 |last1=Smith |first1=Alexandra |last2=Roman |first2=Eve |last3=Howell |first3=Debra |last4=Jones |first4=Richard |last5=Patmore |first5=Russell |last6=Jack |first6=Andrew |author7=Haematological Malignancy Research Network }}</ref>
Diffuse large B cell lymphoma is the most common type of [[non-Hodgkin lymphoma]] among adults, with an annual [[Incidence (epidemiology)|incidence]] of 7–8 cases per 100,000 people per year. This cancer occurs primarily in older individuals, with a median age of diagnosis at approximately 70 years of age, though it can also occur in children and young adults in rare cases.
==Risk factors==
==Risk factors==
According to the American Cancer Society, There are several Factors including Old Age, Male Gender, White Ethnicity, Geography, exposure to Certain Drugs and Chemicals, Radiation exposure, and weak Immune System that can increase the chances of getting Non Hodgkin Lymphoma(NHL) among which Diffuse Large B cell Lymphoma is the Most common sub-type. Some Other Risk Factors include hepatitis C virus (HCV) seropositivity, high body mass as a young adult, HIV, and Family History of Lymphoma
According to the American Cancer Society, There are several Factors including Old Age, Male Gender, White Ethnicity, Geography, exposure to Certain Drugs and Chemicals, Radiation exposure, and weak Immune System that can increase the chances of getting [[Non Hodgkin Lymphoma(NHL)]] among which Diffuse Large B cell Lymphoma is the Most common sub-type. Some Other Risk Factors include [[Hepatitis C virus (HCV) cis-acting replication element (CRE)|hepatitis C virus (HCV]]) [[seropositivity,]] high body mass as a young adult, HIV, and Family History of Lymphoma
==Screening==
==Screening==
Screening for diffuse large B cell lymphoma is not recommended.
Screening for diffuse large B cell lymphoma is not recommended.
Line 31: Line 31:
Laboratory tests of diffuse large B cell lymphoma include [[complete blood count]], blood chemistry studies, [[flow cytometry]], [[immunohistochemistry]], [[cytology]], [[genetic testing]], and [[immunophenotyping]].
Laboratory tests of diffuse large B cell lymphoma include [[complete blood count]], blood chemistry studies, [[flow cytometry]], [[immunohistochemistry]], [[cytology]], [[genetic testing]], and [[immunophenotyping]].
===Electrocardiogram===
===Electrocardiogram===
There are no electrocardiogam findings associated with diffuse large B cell lymphoma.
There are no [[electrocardiogam]] findings associated with diffuse large B cell lymphoma.
===Chest X ray===
===Chest X ray===
There are no chest X ray findings associated with diffuse large B cell lymphoma.
There are no chest X ray findings associated with diffuse large B cell lymphoma.
===CT===
===CT===
[[CT]] scan may be helpful in the diagnosis of diffuse large B cell lymphoma.
CT scan may be helpful in the diagnosis of diffuse large B cell lymphoma. Fluorodeoxyglucose positron emission tomography (FDG-PET)/computed tomography (CT) scan is now recommended as the gold standard for staging DLBCL patients
===MRI===
===MRI===
For suspected lymphoma of the Nervous System, magnetic resonance imaging (MRI) is the modality of choice. Neck MRI is helpful in the diagnosis of diffuse large B cell lymphoma.
For suspected lymphoma of the Nervous System, [[magnetic resonance imaging (MRI)]] is the modality of choice. Neck MRI is helpful in the diagnosis of diffuse large B cell lymphoma.
 
===Biopsy===
===Biopsy===
Lymph node or extranodal tissue [[biopsy]] is diagnostic of large B cell lymphoma.
Lymph node or extranodal tissue [[biopsy]] is diagnostic of large B cell lymphoma.
Line 49: Line 50:
===Surgery===
===Surgery===
Surgical intervention is not recommended for the management of diffuse large B cell lymphoma.
Surgical intervention is not recommended for the management of diffuse large B cell lymphoma.
=== Primary prevention ===
There are no primary preventive measures for diffuse large B cell lymphoma.
=== Secondary prevention ===
There are no secondary preventive measures for diffuse large B cell lymphoma.
===Future or Investigational therapies===
===Future or Investigational therapies===
Active areas of research include genetic studies to assess the genetic role in diffuse large B cell lymphoma and novel therapeutic regimens that evaluate the efficacy and safety of novel regimens in in newly diagnosed diffuse large B cell lymphoma.
Active areas of research include genetic studies to assess the genetic role in diffuse large B cell lymphoma and novel therapeutic regimens that evaluate the efficacy and safety of novel regimens in in newly diagnosed diffuse large B cell lymphoma.

Latest revision as of 19:36, 22 January 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2] Anila Hussain, MD [3]

Overview

Diffuse large B-cell lymphoma (DLBCL or DLBL) is a cancer of B cells, a type of white blood cell responsible for producing antibodies. Diffuse large B cell lymphoma may be classified based on location into nodal and extranodal disease and based on molecular, genetic, and immunohistochemical features into more than 20 subgroups. Diffuse large B cell lymphoma may be classified into 2 subtypes based on gene expression profiles. The progression to diffuse large B cell lymphoma involves the microRNAs (miRNAs). On microscopic histopathological analysis, diffuse large B cell lymphoma can be divided into three variants: centroblastic, immunoblastic, and anaplastic. There are no established causes for diffuse large B cell lymphoma. Diffuse large B cell lymphoma is the most common type of non-Hodgkin lymphoma among adults, with an annual incidence of 7–8 cases per 100,000 people per year. This cancer occurs primarily in older individuals, with a median age of diagnosis at approximately 70 years of age, though it can also occur in children and young adults in rare cases. The most potent risk factor in the development of diffuse large B cell lymphoma is immunodeficiency. Diffuse large B cell lymphoma must be differentiated from other diseases such as follicular lymphoma, Mucosa-Associated Lymphatic Tissue lymphoma(MALT), small cell lymphocytic lymphoma, and Mantle cell lymphoma (MCL). The prognosis of diffuse large B cell lymphoma depends on the stage of the disease. Diffuse large B cell lymphoma is associated with a 5 year survival rate ranging from 70% to more than 90% among children. According to the Lugano classification, there are four stages of diffuse large B cell lymphoma based on the number of nodes involved and extranodal status. The most common symptoms of diffuse large B cell lymphoma include a rapidly enlarging, non-painful mass that may be located in neck, groin or abdomen. The less common symptoms of diffuse large B cell lymphoma include fever, weight loss and drenching night sweats. Common physical examination findings of diffuse large B cell lymphoma include a non tender mass which can arise in virtually any part of the body. Laboratory tests of diffuse large B cell lymphoma include complete blood count, blood chemistry studies, flow cytometry, immunohistochemistry, cytology, genetic testing, and immunophenotyping. CT scan, MRI, and PET may be helpful in the diagnosis of diffuse large B cell lymphoma. Lymph node or extranodal tissue biopsy is diagnostic of large B cell lymphoma. Other diagnostic studies for diffuse large B cell lymphoma include laparoscopy, laparotomy, and bone marrow aspiration and biopsy. The optimal therapy for diffuse large B cell lymphoma depends on the stage at diagnosis. The predominant therapy for diffuse large B cell lymphoma is chemotherapy. Adjunctive radiotherapy may be required. Active areas of research include genetic studies to assess the genetic role in diffuse large B cell lymphoma and novel therapeutic regimens that evaluate the efficacy and safety of novel regimens in in newly diagnosed diffuse large B cell lymphoma.

Classification

Diffuse large B cell lymphoma may be classified based on location into nodal and extranodal disease and based on molecular, genetic, and immunohistochemical features into more than 20 subgroups.

Pathophysiology

Diffuse large B cell lymphoma is mainly caused by genetic mutations. Genetic expression of germinal centers B cell like are associated with favourable prognosis. Some studies have established an association between microRNA expression and B cell lymphoma pathogenesis. The studies showed poor prognosis of microRNA expressed lymphomas. MicroRNAs participate in development of B cell receptor signalling, B cell migration, and class switching of immunoglobulins. On microscopic pathology, diffuse large B cell lymphoma has three variant pictures which include centroblastic, immunoblastic, and anaplastic forms of DLBCL.

Causes

There are no established causes for diffuse large B cell lymphoma.

Differential diagnosis

Diffuse large B cell lymphoma must be differentiated from other diseases such as follicular lymphoma, Mucosa-Associated Lymphatic Tissue lymphoma(MALT), small cell lymphocytic lymphoma, and mantle cell lymphoma (MCL).

Epidemiology and demographics

Diffuse large B cell lymphoma is the most common type of non-Hodgkin lymphoma among adults, with an annual incidence of 7–8 cases per 100,000 people per year. This cancer occurs primarily in older individuals, with a median age of diagnosis at approximately 70 years of age, though it can also occur in children and young adults in rare cases.

Risk factors

According to the American Cancer Society, There are several Factors including Old Age, Male Gender, White Ethnicity, Geography, exposure to Certain Drugs and Chemicals, Radiation exposure, and weak Immune System that can increase the chances of getting Non Hodgkin Lymphoma(NHL) among which Diffuse Large B cell Lymphoma is the Most common sub-type. Some Other Risk Factors include hepatitis C virus (HCV) seropositivity, high body mass as a young adult, HIV, and Family History of Lymphoma

Screening

Screening for diffuse large B cell lymphoma is not recommended.

Prognosis

The prognosis of diffuse large B cell lymphoma depends on the stage of the disease. Diffuse large B cell lymphoma is associated with a 5 year survival rate ranging from 70% to more than 90% among children.

Diagnosis

Staging

According to the Lugano classification, there are four stages of diffuse large B cell lymphoma based on the number of nodes involved and extranodal status.

History and symptoms

The most common symptoms of diffuse large B cell lymphoma include a rapidly enlarging, non-painful mass that may be located in neck, groin or abdomen. The less common symptoms of diffuse large B cell lymphoma include fever, weight loss and drenching night sweats.

Physical examination

Common physical examination findings of diffuse large B cell lymphoma include a non tender mass which can arise in virtually any part of the body.

Laboratory Findings

Laboratory tests of diffuse large B cell lymphoma include complete blood count, blood chemistry studies, flow cytometry, immunohistochemistry, cytology, genetic testing, and immunophenotyping.

Electrocardiogram

There are no electrocardiogam findings associated with diffuse large B cell lymphoma.

Chest X ray

There are no chest X ray findings associated with diffuse large B cell lymphoma.

CT

CT scan may be helpful in the diagnosis of diffuse large B cell lymphoma. Fluorodeoxyglucose positron emission tomography (FDG-PET)/computed tomography (CT) scan is now recommended as the gold standard for staging DLBCL patients

MRI

For suspected lymphoma of the Nervous System, magnetic resonance imaging (MRI) is the modality of choice. Neck MRI is helpful in the diagnosis of diffuse large B cell lymphoma.

Biopsy

Lymph node or extranodal tissue biopsy is diagnostic of large B cell lymphoma.

Other Imaging Studies

PET scan may be helpful in the diagnosis of diffuse large B cell lymphoma.

Other Diagnostic Studies

Other diagnostic studies for diffuse large B cell lymphoma include laparoscopy, laparotomy, and bone marrow aspiration and biopsy.

Treatment

Medical therapy

The optimal therapy for diffuse large B cell lymphoma depends on the stage at diagnosis. The predominant therapy for diffuse large B cell lymphoma is chemotherapy. Adjunctive radiotherapy may be required.

Surgery

Surgical intervention is not recommended for the management of diffuse large B cell lymphoma.

Primary prevention

There are no primary preventive measures for diffuse large B cell lymphoma.

Secondary prevention

There are no secondary preventive measures for diffuse large B cell lymphoma.

Future or Investigational therapies

Active areas of research include genetic studies to assess the genetic role in diffuse large B cell lymphoma and novel therapeutic regimens that evaluate the efficacy and safety of novel regimens in in newly diagnosed diffuse large B cell lymphoma.

References


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