Characteristics of stromal cell lymphoma
Set-cell lymphoma is a type of specialized B-cell lymphoma, one of the non-Hodgkin lymphomas, accounting for approximately 6% of all non-Hodgkin lymphomas. It has all the malignant features of both inert and aggressive lymphomas, is more aggressive, has a late clinical stage with extensive extra-nodal infiltration, is insensitive to conventional radiotherapy, and has a poor prognosis.
Clinical presentation
Inert and classic types of lymphoma are classified. Inert lymphoma: The clinical progression is slow, and most of the patients are asymptomatic, with peripheral lymphocytosis and splenomegaly as the main manifestations, usually without obvious lymph node enlargement and generally in good health. Classic set cell lymphoma has an aggressive presentation, most commonly with enlarged lymph nodes and often with systemic symptoms. Almost 70% of patients have stage III or IV disease at the time of diagnosis, often with bone marrow and peripheral blood infiltration. Extra-nodal organs may be invaded, and gastrointestinal invasion is particularly important in the recognition of the disease. Patients with lymphomatous polyp lesions in the large intestine frequently have condylomatous lymphoma. Patients with gastrointestinal invasion frequently have pharyngeal lymphatic ring invasion.
The staging of non-Hodgkin’s lymphoma is followed.
Stage I lesions are limited to 1 lymph node area or involvement of a single extra-nodal organ (IE).
Stage II lesions involve two or more lymph node areas ipsilateral to the diaphragm, or lesions are limited to extra-lymph node organs and more than one lymph node area ipsilateral to the diaphragm (IIE).
Stage III lymphadenopathy is present both above and below the diaphragm. It may be associated with splenic involvement (IIIS), limited extra-nodal organ involvement (IIIE), or splenic and limited extra-nodal organ involvement (IIISE).
Stage IV has extensive disseminated invasion of one or more extra-nodal organs with or without lymph node enlargement. Any involvement of the liver or bone marrow is considered stage IV.
Group A: No systemic symptoms.
Group B: systemic symptoms including unexplained fever (>38°C for three days) or night sweats or weight loss (more than 10% in 6 months).
Treatment of staphylocytic lymphoma
The treatment of staphylocytic lymphoma is determined by clinical stage
For stage I-II patients systemic chemotherapy ± radiation therapy can prolong disease-free survival with a tendency to improve survival.
For stage III-IV patients, systemic chemotherapy is the mainstay, but the traditional CHOP regimen has low efficiency and requires high-intensity chemotherapy.
Commonly used chemotherapy regimens
HyperCVAD/MA regimen is a very intense chemotherapy originally used to treat acute lymphoblastic leukemia, and in combination with rituximab it is a successful regimen for the treatment of laparoscopic lymphoma. It was able to prolong disease-free survival and overall survival. The regimen is: rituximab + cyclophosphamide + epothilone + vincristine + dexamethasone alternating with another regimen: high-dose methotrexate + cytarabine. This regimen is more toxic, including myelosuppression, cardiotoxicity, and neurotoxicity, and requires close testing and aggressive symptomatic management.
The alternating R-CHOP/R-DHAP regimen is also an effective treatment regimen for sarcoid lymphoma: Rituximab + cyclophosphamide + epothilone + vincristine + prednisone for R-CHOP, and rituximab + cyclophosphamide + cytarabine + dexamethasone for R-DHAP.
Rituximab + cytarabine, or rituximab + cytarabine + bendamustine also have some efficiency.
Elderly patients or those with chronic disease who are not suitable for high-intensity chemotherapy regimens may choose rituximab maintenance therapy, or low-intensity MOP or CHOP regimens chemotherapy to improve quality of life.
Treatment of relapsed or refractory regenerative lymphoma
Since there is still no definitive curable solution for lymphoma, most patients will progress to relapse or develop refractory cases. Patients with relapsed/refractory disease may improve survival by adopting the first-line treatment strategy for primary patients, i.e., autologous stem cell transplantation after induction of the HyperCVAD/MA±R regimen. Or participate in clinical trials with newer agents.
New drugs for the treatment of condylomatous lymphoma
The proteasome inhibitor bortezomib, initially used in the treatment of multiple myeloma, is now approved for the treatment of relapsed/refractory condylomatous lymphoma. The targeted drug timsirolimus is used to treat MCLt45I. timsirolimus is an mTOR inhibitor. mTOR is a serine/threonine kinase that controls cellular protein synthesis, angiogenesis, and the cell cycle. By inhibiting mTOR, it can cause cell cycle arrest, inhibit tumor cell proliferation, and have anti-vascular gene effect to reduce tumor vascular density. Clinical application indicates that this drug has significant remission effect on first-line chemotherapy relapse or refractory MCL.
Thalidomide, ralidomide, and the new alkylating agent bendamustine have also been used in the treatment of MCL.