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    Home > Active Ingredient News > Blood System > Professor Yi Shuhua: Diagnosis and Treatment of Lymphoma in the Marginal Zone of the Spleen|The 4th China Conference on Chronic Lymphocytic Leukemia

    Professor Yi Shuhua: Diagnosis and Treatment of Lymphoma in the Marginal Zone of the Spleen|The 4th China Conference on Chronic Lymphocytic Leukemia

    • Last Update: 2021-04-19
    • Source: Internet
    • Author: User
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    At the 4th China Chronic Lymphocytic Leukemia Conference and the 1st China Chronic Lymphocytic Leukemia Working Group (cwCLL) and International Chronic Lymphocytic Leukemia Working Group (iwCLL) Joint Conference, Professor Yi Shuhua from the Hospital of Hematology, Chinese Academy of Medical Sciences Everyone introduced the diagnosis and treatment of Splenic Marginal Zone Lymphoma (SMZL).
    Yimaitongxian is organized as follows for readers.

    The incidence and characteristics of SMZL In China, the incidence of marginal zone lymphoma (MZL) ranks second among all B-cell lymphomas, accounting for about 10% of B-cell lymphomas.

    SMZL accounts for approximately 20% of MZL (approximately 2% of B-cell lymphoma).

    Professor Yi Shuhua said that domestic and foreign data show that SMZL occurs more frequently in the elderly, with a median diagnosis age of 65, and there is no significant difference in the proportion of males and females.

    Most patients have no obvious clinical symptoms or have an indolent course.

    The main clinical manifestations of advanced SMZL are splenomegaly, bone marrow and peripheral blood infiltration.

    In addition, about 20% of patients will show autoimmune symptoms.

    Although the overall prognosis is good, about 10%-20% of patients will develop high-grade lymphoma, the most common being diffuse large B-cell lymphoma (DLBCL).

    The diagnosis of SMZL Professor Yi Shuhua mentioned that the diagnosis of SMZL should at least meet the following conditions: First, the spleen pathology meets the characteristics of SMZL and the Matutes score of the cellular immune phenotype is ≤2 points (Matutes score is the British team of the Royal Marsden Hospital in 1994 A scoring system based on 5 membrane markers proposed in 2005 [see figure below]).

    Matutes score secondly, if the patient is unable to undergo a spleen biopsy, the diagnosis can also be made based on the typical morphological characteristics of peripheral blood or bone marrow combined with cellular immune phenotype and bone marrow pathology with CD20 positive cells in sinus invasion.

    The morphological characteristics of SMZL should be noted that SMZL needs to be differentiated from B-cell chronic lymphoproliferative disease (B-CLPD).

    B-CLPD is a group of mature B-cell clonal proliferative diseases involving peripheral blood and bone marrow.
    Its diagnosis and differential diagnosis have always been difficult points in clinical work.

    Because SMZL has a certain similarity with other B-CLPD, it is easy to be misdiagnosed and missed.

    Professor Yi Shuhua introduced that at this stage, through systematic flow cytometry immunophenotyping analysis combined with cytogenetics and molecular biology examinations, most B-CLPD can be diagnosed and differentially diagnosed.

    The prognosis of SMZL.
    Fortunately, most of the clinical process of SMZL patients is inert and the prognosis is good.

    The median survival time of the disease is more than 10 years, and the cause of death of patients is mostly treatment-related adverse reactions or secondary tumors, rather than SMZL disease itself.

    A study of long-term follow-up data of 1298 SMZL patients showed that the 5-year overall survival (OS) rate of SMZL patients was as high as 67.
    9%, and the 10-year OS rate was 41.
    9%.
    The older age and the presence of B symptoms at the first diagnosis are the poor prognosis of SMZL.
    factor.

    The Italian Lymphoma Collaboration Group also conducted a retrospective analysis of 309 SMZL patients.
    The results showed that the 5-year cancer-specific survival (CSS) rate of SMZL patients was 76%, hemoglobin was less than 120g/L, and lactate dehydrogenase (LDH) was high.
    The upper limit of normal and albumin <35g/L are poor prognostic factors for OS and CSS of SMZL.

    Professor Yi Shuhua introduced that NOTCH2 and TP53 gene mutations are independent risk factors affecting the prognosis of SMZL.

    A multicenter cohort study included 175 SMZL patients to study the detailed genetic background of SMZL patients and explore its clinical significance.
    The results of the study showed that NOTCH2 mutation is an independent risk factor for TTFT (time to first treatment), while TP53 Mutations increase the risk of death by 136%.

    New progress in SMZL treatment Professor Yi Shuhua emphasized that some SMZL patients have no clinical symptoms.

    Early or asymptomatic patients with no B symptoms, no obvious splenomegaly, no or mild cytopenia, no severe lymph node disease, etc.
    can wait for observation, and perform a comprehensive physical examination every 3-6 months to monitor the condition.

    But about 70% of patients will eventually need treatment.

    01 First-line treatment It should be noted that for SMZL patients with HCV (hepatitis C virus) infection, antiviral therapy can be used as a first-line treatment.

    Splenectomy is the main treatment method for SMZL before the advent of rituximab, which can reduce the burden of disease, improve abdominal discomfort and blood cell reduction.
    At the same time, splenectomy can alleviate the symptoms of anemia and thrombocytopenia in patients with SMZL.

    After first-line splenectomy, the patient’s median progression-free survival (PFS) was 8.
    25 years, and the 5-year OS rate was 84%.

    Therefore, Professor Yi Shuhua said: For patients who are eligible for splenectomy, splenectomy is still necessary.
    However, splenectomy can only improve symptoms.
    When the patient has severe bone marrow or other infiltration, lymphadenopathy, and general When the condition is poor and cannot tolerate surgery, splenectomy is no longer suitable as the preferred method.

    The application of rituximab further increases the remission rate of SMZL, which has become the basis for the treatment of SMZL patients.

    Systemic treatment should be selected when recurrence after splenectomy or splenectomy is not suitable, and rituximab combined or not combined with chemotherapy drugs can be selected.

    The remission rate, duration of remission and safety of rituximab in the first-line treatment of SMZL are superior to those of splenectomy.

    Research data shows that the effective rate of rituximab as an induction therapy for SMZL is as high as 91%, and as a maintenance therapy, it can significantly delay disease progression.

    02 Second-line treatment In addition to traditional chemotherapeutics and rituximab, there are currently many new chemotherapeutic drugs and immune preparations undergoing clinical trials.
    The development and progress of new drugs for B-cell lymphoma have also further broadened the treatment thinking of SMZL: the guidelines recommend Various types of new targeted drugs represented by BTK inhibitors are used in the second-line treatment of MZL; bortezomib in the second-line treatment of MZL and other indolent lymphomas has a good overall remission; lenalidomide combined with rituximab in the second-line treatment of MZL significantly improves the overall Remission rate (ORR) and complete remission (CR) rate reduce the risk of disease progression or death by 54%; PI3Kδ inhibitor Idelalisib as a single agent in the treatment of relapsed/refractory MZL also shows a good effect.

    03 Other treatment methods Professor Yi Shuhua also said that other new treatment options such as local radiotherapy, autologous hematopoietic stem cell transplantation (ASCT), chimeric antigen receptor (CAR) T cell therapy and other treatments in MZL are also actively trying.

    Finally, Professor Yi Shuhua mentioned that the diagnosis and treatment of MZL are still full of challenges.

    First, it is still challenging to identify the risk of early recurrence and/or poor prognosis, and a new clinical genetic risk stratification model is required; second, limited by sample size and clinical plan design, there is a lack of targeted clinical research on MZL or even SMZL; In the context of rapid progress in targeted therapy, the overall progress of MZL has been slow, and there is no standard treatment.

    Although the treatment of MZL has made progress in recent years, and there are more and more targeted therapies, there is still no cure for MZL other than hematopoietic stem cell transplantation, which highlights the need to explore new combinations and consolidation treatments.

    Professor Yi Shuhua Doctor of Medicine, Associate Chief Physician, Master's Tutor, Hematology Hospital of Chinese Academy of Medical Sciences (Institute of Hematology), Member of the 11th Committee of Lymphocytic Diseases Group of Hematology Branch of Chinese Medical Association, China Anti-Cancer Association Hematology Oncology Major Committee Youth Member, Secretary-General, Chinese Society of Immunology, Member of the Hematology and Immunology Branch of the Chinese Society Hope National Medical Center postdoctoral stamp "read the original", we make progress together
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