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Epstein-Barr virus (EBV)-related lymphoid tissue proliferative diseases are a group of diseases with large differences in severity
.
With the promotion of new coronavirus research, the research of EBV-related lymphoid tissue proliferative diseases has also ushered in a new climax, which has attracted widespread attention at home and abroad
.
In order to further discuss the diagnosis, treatment and development of lymphoma in China, a series of activities of the Lymphocytic Disease Group of the Hematology Branch of the Chinese Medical Association-the 2nd Bethune Lymphoma Youth Forum in 2021 will be held online on May 29, 2021
.
At this meeting, Professor Wang Zhao from Beijing Friendship Hospital affiliated to Capital Medical University gave a theme report on "EBV-related Lymphoid Tissue Proliferative Disease (EBV-LPD) Diagnosis and Treatment".
Yimaitong organized the main contents as follows
.
EBV and related diseases Professor Wang Zhao first introduced EBV
.
EBV is a common and highly infectious virus in the human body.
About 90% of people have latent infection with EBV
.
Studies have found that primary hemophagocytic syndrome (HLH) is partly driven by EBV
.
Relevant genetic studies have confirmed that patients with genetic defects are highly susceptible to EBV infection and are difficult to remove for life.
EBV infection may induce HLH and even life-threatening, and such patients are prone to be accompanied by lymphoma
.
About 95% of patients with EBV infection are asymptomatic.
If clinical symptoms such as fever, malaise, and cytopenia are present, it is infectious mononucleosis (leaflet).
Severe leaflets may also be accompanied by severe liver damage, blood cell reduction, etc.
.
Some patients with severe leaflets will develop chronic active EBV infection, which may be accompanied by hemophagocytic syndrome and life-threatening
.
Patients with chronic active EBV infection will eventually undergo clonal proliferation changes, leading to various tumors, including lymphoma, gastric cancer, leiomyosarcoma, and nasopharyngeal carcinoma
.
EBV infection or reactivation is related to immune status, including congenital immunodeficiency, acquired immunodeficiency, and immunodeficiency caused by other viral infections (such as HIV).
In addition, patients who have taken immunosuppressants or hormones for a long time after organ transplantation , Or patients with other malignant tumors, are prone to EBV infection
.
Diagnosis and treatment of EBV-B-LPD EBV-related B-cell lymphoid tissue proliferative disease (EBV-B-LPD) is a disease caused by EBV infecting only B cells through CD19, including post-transplant lymphoid tissue proliferative diseases and some lymphomas
.
Compared with other diseases caused by EBV infection, EBV-B-LPD is less difficult to treat and has better curative effect
.
At present, the main treatment options are favorable tuximab, EBV-CTL (EB virus-specific cytotoxic T lymphocytes), CAR-T (chimeric antigen receptor T cells) and chemotherapy
.
Diagnosis and treatment of EBV-T/NK-LPD Professor Wang then focused on the EBV-related T/NK cell lymphoid tissue proliferative disease (EBV-T/NK-LPD), which is a type of fatal disease that has attracted much attention in clinical practice.
Disease
.
And through two cases, the clinical diagnosis and treatment of EBV-T/NK-LPD were explained in a simple and profound way
.
Classification and diagnosis of EBV-T/NK-LPD EBV-T/NK-LPD can be divided into three types: hemophagocytic syndrome, chronic active EBV infection and malignant tumors
.
Chronic active EBV infection (CAEBV) is more common in children and adolescents.
Adults have a lower incidence, but the prognosis of adults is poor, and adolescents tend to survive longer
.
Skin-type CAEBV has a better prognosis than systemic CAEBV and has a longer survival time.
Therefore, the transplant period of such patients requires more evaluation, rather than transplantation as soon as the diagnosis is made
.
Invasive NK-cell leukemia (ANKL) is a rare systemic mature NK-cell proliferative tumor with an aggressive and explosive clinical course, with a median survival time of less than 2 months
.
The age of onset of patients is mostly 30-50 years old, with no gender difference.
Among them, pediatric patients are mostly caused by the progression of other types of EBV-LPD
.
Regarding the diagnosis of EBV-related diseases, Professor Wang emphasized that there are several points that need special attention: First, the phenomenon of hemophagocytosis is not only in the bone marrow, but also includes other tissue biopsies
.
The clinical examination should be comprehensive to avoid the delay in the diagnosis of hemophagocytosis.
Second, monoclonal TCR rearrangements and heavy chain gene rearrangements exist not only in tumor patients, but also in patients with chronic active EBV infection or leaflets.
Therefore, they cannot As a basis for tumor diagnosis (especially in the case of EBV positive)
.
In addition, in clinicopathological diagnosis, leaflets are sometimes misdiagnosed as lymphoma.
This is because leaflets infect B cells, but T cells are the response to proliferation.
After a large number of excessive proliferation of T cells, it is highly similar to lymphoma
.
Therefore, you need to be more cautious when diagnosing
.
At present, through EBER-ISH and CD79a or CD3 double staining, the types of EBV-infected cells can be judged, so as to distinguish between EBV-B-LDP and EBV-T/NK-LPD, and guide clinical treatment options
.
In addition, genetic screening can also help clinical diagnosis and treatment
.
Professor Wang especially emphasized that the research results have proved that CD27 and CD70 are closely related to primary HLH
.
Patients with EBV-LPD and EBV-associated lymphoma must undergo genetic screening.
If there are CD27 and CD70 gene defects, the best treatment option is to undergo allogeneic hematopoietic stem cell transplantation (allo-HSCT) as soon as possible, rather than chemotherapy or autologous transplantation.
These two treatment options can only benefit in the short term
.
In addition, there is a strong correlation between the 4-1BB defect and EBV-LPD
.
Treatment of EBV-T/NK-LPD At present, there is no standard treatment plan for EBV-NK/T-LPD.
EBV-infected T/NK cells are often resistant to chemotherapy.
Allo-HSCT is currently the only cure for the disease
.
In terms of drug treatment, the efficacy of rocotinib monotherapy and L-DEP in children is better than that in adult patients.
Some children can even be cured.
Adult patients need allo-HSCT treatment in the later stage
.
Regarding the timing of transplantation, due to the large clinical heterogeneity of EBV-NK/T-LPD, some patients have no therapeutic intervention, and the disease can remain stable for a period of time, while some patients have rapid disease progression and severe comorbidities in a short period of time.
Therefore, the timing of treatment of allo-HSCT is not clear
.
Currently there are many non-allo-HSCT regimen being explored, including: immunization checkpoint inhibitor (PD-1), JAK2 inhibitors, proteasome inhibitors and the like
.
For some patients who cannot be transplanted, the donor's stem cell or lymphocyte infusion can be used to achieve a transient curative effect, but allo-HSCT treatment is still needed in the later stage
.
In addition, BARF1-specific T cell therapy has good prospects, but there are still many difficulties
.
Summary Generally speaking, with the in-depth study of genetics, we have a deeper understanding of EBV-related lymphoid tissue proliferative diseases, but there are still many problems in the pathogenesis and diagnosis that need to be solved urgently
.
In terms of treatment, for EBV-B-LPD, rituximab has a definite effect, while there is no standard treatment plan for EBV-NK/T-LPD.
Allo-HSCT is currently the only method that can cure the disease.
The non-transplantation treatment plan is currently Still exploring
.
Professor Wang Zhao, Chief Physician, PhD Student (Post) Supervisor, Director of the Department of Hematology, Beijing Friendship Hospital, Capital Medical University, Executive Member of the International Tissue Cell Association Hemophagocytic Syndrome, Chairman of the Tissue Cell Disease Professional Committee of the Chinese Medical Doctor Association Hematologists Branch Chinese Medicine Deputy Leader of the Lymphocytic Disease Group of the Society of Hematology, Member of the Standing Committee of the Hematology Committee of the Chinese Society of Immunology, Member of the Standing Committee of the Lymphoma Professional Committee of the Chinese Anti-Cancer Association, Member of the Standing Committee of the Chinese Anticancer Association Hematological Translational Medicine Professional Committee Member of the Standing Committee of the Sub-Committee, Member of the Standing Committee of the Professional Committee of Cord Blood Clinical Application of China Maternal and Child Health Association, stamped "Read the original", and we will make progress together
.
With the promotion of new coronavirus research, the research of EBV-related lymphoid tissue proliferative diseases has also ushered in a new climax, which has attracted widespread attention at home and abroad
.
In order to further discuss the diagnosis, treatment and development of lymphoma in China, a series of activities of the Lymphocytic Disease Group of the Hematology Branch of the Chinese Medical Association-the 2nd Bethune Lymphoma Youth Forum in 2021 will be held online on May 29, 2021
.
At this meeting, Professor Wang Zhao from Beijing Friendship Hospital affiliated to Capital Medical University gave a theme report on "EBV-related Lymphoid Tissue Proliferative Disease (EBV-LPD) Diagnosis and Treatment".
Yimaitong organized the main contents as follows
.
EBV and related diseases Professor Wang Zhao first introduced EBV
.
EBV is a common and highly infectious virus in the human body.
About 90% of people have latent infection with EBV
.
Studies have found that primary hemophagocytic syndrome (HLH) is partly driven by EBV
.
Relevant genetic studies have confirmed that patients with genetic defects are highly susceptible to EBV infection and are difficult to remove for life.
EBV infection may induce HLH and even life-threatening, and such patients are prone to be accompanied by lymphoma
.
About 95% of patients with EBV infection are asymptomatic.
If clinical symptoms such as fever, malaise, and cytopenia are present, it is infectious mononucleosis (leaflet).
Severe leaflets may also be accompanied by severe liver damage, blood cell reduction, etc.
.
Some patients with severe leaflets will develop chronic active EBV infection, which may be accompanied by hemophagocytic syndrome and life-threatening
.
Patients with chronic active EBV infection will eventually undergo clonal proliferation changes, leading to various tumors, including lymphoma, gastric cancer, leiomyosarcoma, and nasopharyngeal carcinoma
.
EBV infection or reactivation is related to immune status, including congenital immunodeficiency, acquired immunodeficiency, and immunodeficiency caused by other viral infections (such as HIV).
In addition, patients who have taken immunosuppressants or hormones for a long time after organ transplantation , Or patients with other malignant tumors, are prone to EBV infection
.
Diagnosis and treatment of EBV-B-LPD EBV-related B-cell lymphoid tissue proliferative disease (EBV-B-LPD) is a disease caused by EBV infecting only B cells through CD19, including post-transplant lymphoid tissue proliferative diseases and some lymphomas
.
Compared with other diseases caused by EBV infection, EBV-B-LPD is less difficult to treat and has better curative effect
.
At present, the main treatment options are favorable tuximab, EBV-CTL (EB virus-specific cytotoxic T lymphocytes), CAR-T (chimeric antigen receptor T cells) and chemotherapy
.
Diagnosis and treatment of EBV-T/NK-LPD Professor Wang then focused on the EBV-related T/NK cell lymphoid tissue proliferative disease (EBV-T/NK-LPD), which is a type of fatal disease that has attracted much attention in clinical practice.
Disease
.
And through two cases, the clinical diagnosis and treatment of EBV-T/NK-LPD were explained in a simple and profound way
.
Classification and diagnosis of EBV-T/NK-LPD EBV-T/NK-LPD can be divided into three types: hemophagocytic syndrome, chronic active EBV infection and malignant tumors
.
Chronic active EBV infection (CAEBV) is more common in children and adolescents.
Adults have a lower incidence, but the prognosis of adults is poor, and adolescents tend to survive longer
.
Skin-type CAEBV has a better prognosis than systemic CAEBV and has a longer survival time.
Therefore, the transplant period of such patients requires more evaluation, rather than transplantation as soon as the diagnosis is made
.
Invasive NK-cell leukemia (ANKL) is a rare systemic mature NK-cell proliferative tumor with an aggressive and explosive clinical course, with a median survival time of less than 2 months
.
The age of onset of patients is mostly 30-50 years old, with no gender difference.
Among them, pediatric patients are mostly caused by the progression of other types of EBV-LPD
.
Regarding the diagnosis of EBV-related diseases, Professor Wang emphasized that there are several points that need special attention: First, the phenomenon of hemophagocytosis is not only in the bone marrow, but also includes other tissue biopsies
.
The clinical examination should be comprehensive to avoid the delay in the diagnosis of hemophagocytosis.
Second, monoclonal TCR rearrangements and heavy chain gene rearrangements exist not only in tumor patients, but also in patients with chronic active EBV infection or leaflets.
Therefore, they cannot As a basis for tumor diagnosis (especially in the case of EBV positive)
.
In addition, in clinicopathological diagnosis, leaflets are sometimes misdiagnosed as lymphoma.
This is because leaflets infect B cells, but T cells are the response to proliferation.
After a large number of excessive proliferation of T cells, it is highly similar to lymphoma
.
Therefore, you need to be more cautious when diagnosing
.
At present, through EBER-ISH and CD79a or CD3 double staining, the types of EBV-infected cells can be judged, so as to distinguish between EBV-B-LDP and EBV-T/NK-LPD, and guide clinical treatment options
.
In addition, genetic screening can also help clinical diagnosis and treatment
.
Professor Wang especially emphasized that the research results have proved that CD27 and CD70 are closely related to primary HLH
.
Patients with EBV-LPD and EBV-associated lymphoma must undergo genetic screening.
If there are CD27 and CD70 gene defects, the best treatment option is to undergo allogeneic hematopoietic stem cell transplantation (allo-HSCT) as soon as possible, rather than chemotherapy or autologous transplantation.
These two treatment options can only benefit in the short term
.
In addition, there is a strong correlation between the 4-1BB defect and EBV-LPD
.
Treatment of EBV-T/NK-LPD At present, there is no standard treatment plan for EBV-NK/T-LPD.
EBV-infected T/NK cells are often resistant to chemotherapy.
Allo-HSCT is currently the only cure for the disease
.
In terms of drug treatment, the efficacy of rocotinib monotherapy and L-DEP in children is better than that in adult patients.
Some children can even be cured.
Adult patients need allo-HSCT treatment in the later stage
.
Regarding the timing of transplantation, due to the large clinical heterogeneity of EBV-NK/T-LPD, some patients have no therapeutic intervention, and the disease can remain stable for a period of time, while some patients have rapid disease progression and severe comorbidities in a short period of time.
Therefore, the timing of treatment of allo-HSCT is not clear
.
Currently there are many non-allo-HSCT regimen being explored, including: immunization checkpoint inhibitor (PD-1), JAK2 inhibitors, proteasome inhibitors and the like
.
For some patients who cannot be transplanted, the donor's stem cell or lymphocyte infusion can be used to achieve a transient curative effect, but allo-HSCT treatment is still needed in the later stage
.
In addition, BARF1-specific T cell therapy has good prospects, but there are still many difficulties
.
Summary Generally speaking, with the in-depth study of genetics, we have a deeper understanding of EBV-related lymphoid tissue proliferative diseases, but there are still many problems in the pathogenesis and diagnosis that need to be solved urgently
.
In terms of treatment, for EBV-B-LPD, rituximab has a definite effect, while there is no standard treatment plan for EBV-NK/T-LPD.
Allo-HSCT is currently the only method that can cure the disease.
The non-transplantation treatment plan is currently Still exploring
.
Professor Wang Zhao, Chief Physician, PhD Student (Post) Supervisor, Director of the Department of Hematology, Beijing Friendship Hospital, Capital Medical University, Executive Member of the International Tissue Cell Association Hemophagocytic Syndrome, Chairman of the Tissue Cell Disease Professional Committee of the Chinese Medical Doctor Association Hematologists Branch Chinese Medicine Deputy Leader of the Lymphocytic Disease Group of the Society of Hematology, Member of the Standing Committee of the Hematology Committee of the Chinese Society of Immunology, Member of the Standing Committee of the Lymphoma Professional Committee of the Chinese Anti-Cancer Association, Member of the Standing Committee of the Chinese Anticancer Association Hematological Translational Medicine Professional Committee Member of the Standing Committee of the Sub-Committee, Member of the Standing Committee of the Professional Committee of Cord Blood Clinical Application of China Maternal and Child Health Association, stamped "Read the original", and we will make progress together
