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    Home > Active Ingredient News > Blood System > Professor Qiu Lugui: Diagnosis and treatment of T-cell chronic lymphoproliferative diseases | The 4th China Chronic Lymphocytic Leukemia Conference

    Professor Qiu Lugui: Diagnosis and treatment of T-cell chronic lymphoproliferative diseases | The 4th China Chronic Lymphocytic Leukemia Conference

    • Last Update: 2021-04-20
    • Source: Internet
    • Author: User
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    March 19-21, 2021, 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 was successfully held in Tianjin .

    At the Chronic Lymphoproliferative Disease Summit Forum on March 21, Professor Qiu Lugui from the Hematology Hospital of the Chinese Academy of Medical Sciences introduced the diagnosis and treatment of T cell chronic lymphoproliferative diseases.
    Yimaitong is now organized as follows for readers .

    Professor Qiu said that T-cell chronic lymphoproliferative disease is a type of indolent T-cell lymphoma that is characterized by peripheral blood and bone marrow infiltration and can be basically diagnosed through peripheral blood and bone marrow tests.

    It is relatively rare in clinical practice, and diagnosis and identification are still difficult points in current clinical work.

    The classification of T-cell/B-cell lymphoma is mainly judged according to the development process of lymphocytes.
    According to the 2016 WHO classification of mature T-cell lymphoma, it is mainly divided into nodular, extranodal, skin-based and leukemia type 4 In the big category, Professor Qiu mainly introduced the diagnosis and treatment of T-lymphocytic leukemia (T-PLL) and large granular lymphocytic leukemia (LGLL) in the leukemia type.

    T-PLL diagnosis and treatment T-PLL is relatively rare, accounting for about 2% of adult mature lymphocytic leukemia.

    The clinical features are high white blood cell (>100×109/L), hepatosplenomegaly, lymphadenopathy, skin erythema, peripheral edema/serous cavity effusion.

    In terms of immunophenotype, usually CD4+CD8-, 25% co-express CD4 and CD8; express TCL1 and have certain characteristics; strongly express CD52.

    Genetics can show 14q11 or 14q32 inversion, 11q22 deletion, MYC amplification, JAK3 mutation (30%-40%), STAT5B mutation (20%-40%).

    Serum HTLV-1 was negative.

    At the same time, Professor Qiu also said that the histopathology of T-PLL is not characteristic, and it is difficult to diagnose bone marrow pathology alone.

    In clinical practice, attention should be paid to the diagnostic value of peripheral blood smear morphology.

    The diagnostic criteria of T-PLL are shown in the figure below.

    Regarding the treatment after diagnosis, Professor Qiu pointed out that not all patients with T-PLL need treatment.

    Active treatment is required for patients with complications related to the following diseases: weakness, night sweats, weight loss, symptomatic lymphadenopathy or fever symptomatic anemia and/or thrombocytopenia, skin infiltration, pleural effusion, or central nervous system involvement.
    Progressive disease , Manifested as a progressive increase in lymphocytes and/or rapid enlargement of lymph nodes, spleen and liver.

    In contrast, transient local lymphadenopathy (response to local infection) is not necessarily an indication for treatment.
    However, there is currently no truly effective treatment for T-PLL.

    Nowadays, alemtuzumab, pentostatin, cladribine, fludarabine, bendamustine, FMC regimen, etc.
    are mostly used in international clinical treatment for induction of remission.
    After remission, allogeneic hematopoietic stem cell transplantation is performed for age-appropriate and conditional patients.

    Professor Qiu pointed out that most T-PLLs are aggressive, but some are inert.

    Compared with the aggressive or subsequent progression type, the indolent type is mostly characterized by low tumor burden, and there is no significant difference from the former two in terms of immunophenotype and cytogenetics.

    However, in terms of survival, the median overall survival (OS) period of initial indolent patients can reach 50 months, which is significantly better than aggressive (12 months) or subsequent progression patients (9 months).

    Therefore, Professor Qiu believes that this kind of indolent T-PLL, namely T-chronic lymphocytic leukemia (T-CLL, the current WHO classification system no longer includes T-CLL), should be regarded as an independent type rather than a variant T-PLL , But it is also necessary to further explore the indicators that distinguish the two.

    Diagnosis and treatment of LGLL Subsequently, Professor Qiu introduced the rare disease LGLL, which accounts for the proportion of chronic lymphocytic proliferative diseases: 2%-5% in Western countries and 5%-6% in Asia.

    It is mainly divided into three categories: T-large granular lymphocytic leukemia (T-LGLL), chronic NK cell polycythemia (CLPD-NK), and aggressive NK cell leukemia (ANKL).

    About one-third of LGLL patients are asymptomatic at first diagnosis, and about two-thirds of patients will develop anemia, splenomegaly, and neutropenia during the progression of the disease.
    Neutropenia is common in Western countries.
    my country The symptoms of anemia are more prominent in patients.

    In terms of cell morphology, large granular lymphocytes are large in size and rich in cytoplasm.
    Among them, there are typical azurophilic granules.
    The nucleus is kidney-shaped or round.
    The absolute count of large granular lymphocytes is usually greater than 0.
    5×109/L.

    In addition, it has certain characteristics in immune phenotype and clonal rearrangement.
    Mutations in STAT3 or STAT5B gene also contribute to the diagnosis of LGLL.

    Therefore, Professor Qiu said that for patients with suspected clinical manifestations of LGLL (see the figure below for the diagnosis process), blood counts and smears should be performed to find large granular lymphocytes, and then flow cytometry analysis, and then according to the immune table Type subdivided T large particles or NK large particles, and for atypical manifestations (such as low LGL counts of about 0.
    5-1x109/L, no evidence of blood cloning, STAT3 not mutated, etc.
    ), further bone marrow biopsy and immunohistochemical examinations are recommended.

    Professor Qiu said that asymptomatic LGLLT can wait for observation.
    For patients with obvious symptoms, the routine treatment process is shown in the figure below.

    Traditional immunosuppressants, purine analogs, hematopoietic growth factors, targeted therapy (alemtuzumab, siplizumab, JAK-3 inhibitors, etc.
    ) and hematopoietic stem cell transplantation are mostly used.

    Finally, Professor Qiu introduced the statistics of the mature T-cell lymphoma (MTCL) database of the Hematology Hospital.

    From June 1992 to December 2019, a total of 430 MTCL patients were admitted, of which 2/3 were aggressive and 1/3 were inert.

    The preliminary analysis of survival showed that the median OS of aggressive MTCL was 24 months, and the 5-year OS rate was 31%; the indolent MTCL patients did not reach the median OS, and the 5-year OS rate was 78%.

    In the T-PLL cohort, the 2-year OS rate reached 50.
    8%.

    Professor Qiu’s team created thalidomide combined with prednisone and methotrexate (TPM regimen) to treat symptomatic LGLL.
    A single-center prospective study showed that the median time to optimal treatment response was 6 months.
    According to the actual analysis, the total remission rate is as high as 96%, the complete remission (CR) rate is 81%, and the 3-year OS and progression-free survival rates are 92% and 86% respectively; and most of the anemia and neutropenia can be significantly recovered within 3 months ; Among patients undergoing flow cytometry, 1/3 obtained MRD negative CR; subgroup analysis further showed that TPM regimen showed similar efficacy in patients with or without simple red blood cell aplastic anemia .

    Professor Qiu pointed out that the TPM program has a significant effect compared with previous immunopreparation treatments.
    At present, a multi-center clinical trial has been launched to further expand the scale of research to verify the therapeutic effect.

    Professor Qiu Lugui, Director, Chief Physician, and Doctoral Supervisor of Lymphoma Center, Hospital of Hematology, Chinese Academy of Medical Sciences, Director of Tianjin Cord Blood Hematopoietic Stem Cell Bank, Member of the International Myeloma Working Group Expert Committee Member, Hematological Oncology Committee of the Chinese Anti-Cancer Association Chairman, Chinese Society of Clinical Oncology Vice Chairman of the Lymphoma Research Alliance Vice Chairman of the Hematology Professional Committee of the Integrative Physician Association Vice Chairman of the Hematology Professional Committee of the Chinese Medical Education Association Chairman of the Hematology Professional Committee of the Tianjin Anti-Cancer Association Chairman of the Standing Committee of the Precision Medicine Branch of the Chinese Medical Biotechnology Association "We make progress together
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