Introduction to mantle cell lymphoma

Introduction

Mantle cell lymphomaAccount for allnon-Hodgkin's lymphoma6% (Non-Hodgkin lymphoma, NHL). Differential diagnosis of mantle cell lymphoma includes other small cell B cell lymphomas. The most common manifestation of mantle cell lymphoma is swollen lymph nodes, often accompanied by systemic symptoms. Blood pathologists based on morphological findings and the tumor is a B-cell lymphoma, making a correct diagnosis of mantle cell lymphoma. As with other subtypes of lymphoma, a suitable biopsy is important. The treatment of mantle cell lymphoma CHOP regimen was not satisfactory, and only a few patients achieved complete remission.

Fundamental contents

Mantle cell lymphoma

Disease profile

Mantle cell lymphoma accounts for 6% of all non-Hodgkin's lymphoma (NHL). This type of lymphoma was previously classified in other subtypes and has only been recognized as an independent disease for more than a decade. Recognizing that these lymphomas have characteristic chromosomal translocation t(11;14), that is, the shift between the immunoglobulin heavy chain gene on chromosome 14 and the Bcl-1 gene on chromosome 11, regularly overexpressing Bcl The -1 protein, the most characteristic is the overexpression of Cyclin D1, thus confirming the existence of this type of lymphoma.

Clinical manifestation

The most common manifestation of mantle cell lymphoma is swollen lymph nodes, often accompanied by systemic symptoms. Almost 70% of patients are diagnosed with stage III or IV disease, often with bone marrow and peripheral blood infiltration. Extranodal organs may be invaded, and gastrointestinal invasion is particularly important for understanding the disease. Patients with lymphoma polyp lesions in the large intestine often have mantle cell lymphoma. Patients with gastrointestinal invasion often have pharyngeal lymphatic invasion and so on.

diagnosis

Blood pathologists based on morphological findings and the tumor is a B-cell lymphoma, making a correct diagnosis of mantle cell lymphoma. As with other subtypes of lymphoma, a suitable biopsy is important. Differential diagnosis of mantle cell lymphoma includes other small cell B cell lymphomas. Special sets of cell lymphomas and small lymphocytic lymphomas have characteristic CD5 expression. Mantle cell lymphoma often has a serrated nucleus.

treatment

The treatment of mantle cell lymphoma CHOP regimen was not satisfactory, and only a few patients achieved complete remission. Enhanced combination chemotherapy regimens (such as HyperCVAD/MTX-AraC and EPOCH) are often associated with younger patients with autologous or allogeneic bone marrow transplantation. Several international clinical trials have shown that combined use of rituximab (R) and chemotherapy have better clinical outcomes, so R-HyperCVAD/MTX-AraC, R-EPOCH, R-CHOP and rituximab are currently recommended. Combined with cladribine and the like as a first-line treatment. Considering hematopoietic stem cell transplantation as a first-line consolidation therapy for young patients.RituximabjointThalidomideIt shows good clinical results and can be used as a second-line treatment. Proteasome inhibitorBortezomib(bortezomib) has shown initial efficacy in the treatment of relapsed mantle cell lymphoma, and can also be used as a second-line treatment.

Prognosis

The 5-year survival rate of mantle cell lymphoma is about 25%. The patients with high IPI scores have only a few 5-year survivors, while those with low IPI scores have a 5-year survival rate of 50%.

New therapy biological immunotherapy

Biotherapeutic technology is an emerging anti-cancer method. The treatment method is to take the blood of the patient, extract the immune cells and culture them in vitro, and then return to the patient after the culture, so that the immune cells can fight the cancer cells.

Biotechnology combined with traditional therapy to improve the remission rate of mantle cell lymphoma and prolong the survival of patients. Autologous hematopoietic stem cell transplantation after high-dose myeloablative and post-chemotherapy should be used as a standard treatment for patients with mantle cell lymphoma, but recurrence after transplantation is the main The problem. If combined with biological immunotherapy, not only can enhance the patient's constitution, improve the quality of life, and clinical treatment shows that the complete response rate of patients in the biotherapy + rituximab group is significantly improved (33% vs 0%, P = 0.003), which One result clearly demonstrates the superiority of biological therapy for mantle cell lymphoma, and this increase in remission rate results in a significant improvement in patient survival.