Overview
Definition
Malignant lymphoma is a general term for a group of malignant tumors that originate in the lymphohematopoietic system.
Strictly speaking, all lymphomas are malignant tumors in their own right, so malignant lymphoma is simply an emphasis on the nature of the lymphoma disease.
Lymphoma can occur in any part of the body, lymph nodes, tonsils, spleen and bone marrow are most likely to be involved.
Types
According to histopathologic changes, lymphomas can be divided into two major groups: Hodgkin’s lymphoma (HL) and non-Hodgkin’s lymphoma (NHL).
Hodgkin’s lymphoma
Also known as Hodgkin’s disease, it originates mainly in the lymph nodes and is characterized by progressive enlargement of the lymph nodes.
The 2016 WHO classification of tumors of the lymphohematopoietic system is currently used, which is divided into two categories: nodular lymphocyte-predominant Hodgkin’s lymphoma and classic Hodgkin’s lymphoma.
Nodular lymphocyte-dominant type accounts for 5% of Hodgkin’s lymphomas and classic type accounts for 95% of Hodgkin’s lymphomas.
Nodular Lymphocyte Predominant Hodgkin Lymphoma (NLPHL)
More than 95% are nodular, with microscopic hyperplasia of a single small lymphocyte with scattered large tumor cells (popcorn-like).
Classical Hodgkin’s lymphoma (CHL)
In China, mixed cell type (MCHL) is the most common type of classical HL, followed by nodular sclerosing type (NSHL), lymphocyte-rich type (LRHL), and lymphocyte-depleted type (LDHL).
Non-Hodgkin’s lymphoma
It is a group of lymphomas with different histologic features and sites of onset that are prone to early distant spread.
The main common non-Hodgkin’s lymphoma subtypes are:
Diffuse large B-cell lymphoma (DLBCL)
Marginal zone lymphoma (MZL)
Marginal zone refers to the structure between the lymphoid follicle and the follicular coat; lymphomas occurring in this area are of B-cell origin and are inert lymphomas. They can be divided into 3 subtypes according to the site of involvement:
Follicular lymphoma (FL)
Mantle cell lymphoma (MCL)
It is clinically common in elderly men and belongs to aggressive lymphoma, accounting for 6%~8% of non-Hodgkin’s lymphoma.
This type develops rapidly, with a median survival of 2 to 3 years and a low rate of complete remission with chemotherapy.
Burkitt’s lymphoma/leukemia (BL)
t(8;14) with MYC gene rearrangement is diagnostic, proliferates extremely rapidly, and is a severe aggressive non-Hodgkin’s lymphoma.
Angioimmunoblastic T-cell lymphoma (AITL)
Anaplastic large cell lymphoma (ALCL)
Peripheral T-cell lymphoma (non-specific) (PTCL)
Mycosis fungoides/Sezary syndrome (MF/SS)
It is an inert lymphoma, which is commonly characterized by myxoid granulomas, and is known as Sezary syndrome if it affects the terminal bloodstream.
Incidence
The age of lymphoma incidence in China is mostly between 30 and 40 years old, with a unimodal distribution.
Data from the World Health Organization GLOBOCAN 2020 shows the following incidence of lymphoma in China:
Hodgkin’s lymphoma
In 2020, there were 6829 new cases of Hodgkin’s lymphoma HL) in China, of which 4506 were male and 2323 were female; and there were 2807 deaths, of which 1865 were male and 942 were female.
Non-Hodgkin’s lymphoma
Causes
Causes
The exact cause of malignant lymphoma is not clear, but it is believed to be related to viral and bacterial infections, defective or suppressed immune function, and heredity.
Viral and bacterial infections
The detection rate of EBV in malignant lymphomas such as NK/T-cell lymphoma, Hodgkin’s lymphoma, and Burkitt’s lymphoma is up to 70% or more, and there are also cases such as gastrointestinal mucosa-associated lymphoid tissue lymphomas which may be associated with Helicobacter pylori infection, so viral-bacterial infections may be related to the development of malignant lymphomas.
Deficiency or suppression of immune function
Malignant lymphoma is a disease originating from the immune system, and immune function deficiency is one of the important reasons for its development. In addition, long-term use of immunosuppressants such as methotrexate and azathioprine will also increase the risk of malignant lymphoma.
Heredity
Research shows that malignant lymphoma can show family aggregation, such as small lymphocytic lymphoma relatives will increase the risk of lymphoma by about 5 times.
Predisposing Factors
The following factors may increase the risk of malignant lymphoma.
Work environment and occupation
Working environment with long-term exposure to chemical agents such as pesticides, herbicides, paints, etc., and people exposed to radioactive substances such as hospital radiology staff and nuclear power plant workers.
Age
The two peaks of malignant lymphoma incidence are 15-30 years old and over 55 years old, especially middle-aged and elderly men, the risk of malignant lymphoma will increase year by year.
Pathogenesis
The exact pathogenesis of lymphoma is not well understood and may be related to the following mechanisms:
Symptoms
Lymphocytes can undergo malignant transformation in their “birthplace” (thymus, bone marrow) as well as in lymph nodes, spleen, tonsils and lymphoid tissues of other tissues and organs throughout the body.
Advanced malignant lymphoma can also invade parts other than lymphatic tissues, and the clinical manifestations of lymphoma are complicated and diversified.
This entry only briefly describes the main symptoms, for more symptoms, please refer to reading Lymphoma.
Main Symptoms
Localized symptoms
Systemic symptoms
Complications
Leukemia.
Proliferating lymphocytes enter the bone marrow in the later stages of the disease, causing more than 20% of lymphoma cells in the bone marrow is called lymphomatous leukemia, and the condition needs to be confirmed by a bone marrow smear biopsy.
Anemia
When malignant lymphoma invades the bone marrow, the bone marrow proliferation is inhibited, and the patient will have anemia; invading the gastric mucosa and causing gastrointestinal bleeding is also one of the causes of anemia.
Medical treatment
Department of Medicine
Department of Hematology
Malignant lymphoma is a disease of the hematopoietic system and can be treated in hematology or hematology.
Oncology
The Department of Oncology can provide chemotherapy, radiotherapy and targeted therapy for malignant tumors, and develop targeted treatment plans.
Gastroenterology
Some gastrointestinal lymphomas may present with symptoms such as abdominal pain and bloating, loss of appetite, etc. They can be treated in the Department of Gastroenterology or Gastroenterology.
Preparation for medical treatment
Consultation: Registration, Preparation of documents, Frequently Asked Questions
Tips for the doctor
Wear loose-fitting clothing to facilitate palpation of superficial lymph nodes throughout the body.
Preparation Checklist
Symptom list
Pay particular attention to the time of onset of symptoms, special manifestations, etc.
Medical History Checklist
Checklist
Test results in the past six months, which can be brought to the doctor’s office
Diagnosis
Diagnosis is based on
Medical history
The patient may have a history of the following:
Clinical manifestations
Patients may have the following clinical manifestations:
Symptoms
Physical signs
Staging
Most types of lymphoma are staged with reference to the 2014 Lugano staging criteria.
Limited stage
Stage I.
Invasion of only a single lymph node region (I), or invasion of a single extranodal organ without lymph node involvement (IE).
Stage II
Invasion of ≥2 lymph node regions, but all ipsilateral to the diaphragm (II), which may be accompanied by limited extranodal organ involvement in the ipsilateral lymph node drainage region (IIE).
Stage II large masses, i.e., those with large masses in stage II, are usually the staging test for CT, MRI, or PET/CT.
Progressive stage
Stage III.
Invasion of the lymph node region above and below the diaphragm, or invasion of the supradiaphragmatic lymph nodes + splenic involvement (IIIS).
Stage IV
Invasion of extranodal organs beyond the lymph node drainage area (IV).
Differential diagnosis
Lymphadenitis
Necrotizing lymphadenitis and tuberculous lymphadenitis are common.
Infectious mononucleosis
Tonsillitis
Treatment
Tips: Different types of lymphoma have different treatment plans, this article only briefly describes the main treatment means, please refer to the article of each subtype of lymphoma for specific plans.
Chemotherapy
Chemotherapy is the main treatment for lymphoma. Chemotherapy for lymphoma mostly adopts multi-drug combination chemotherapy regimen. According to the characteristics of the growth cycle of lymphoma cells, cell cycle specific and cell cycle non-specific drugs are combined.
Commonly used chemotherapy drugs for lymphoma
Characteristics of chemotherapy for lymphoma
Compared with solid tumors, the chemotherapy dose for lymphoma is larger, the treatment time is longer, and the patient’s general condition is relatively poorer, so it is necessary to closely monitor and promptly deal with the toxicity of myelosuppression, cardiac injury, liver and kidney damage, etc. caused by chemotherapy.
Lymphoma patients are more common among adolescents and have a higher cure rate, so the long-term toxicity caused by chemotherapy (e.g., infertility, second tumors) is also getting more attention.
Doctors usually combine chemotherapeutic agents to minimize toxic side effects while ensuring efficacy.
Commonly used chemotherapy regimens
Radiation therapy
Lymphoma is one of the most common radiosensitive tumors and radiotherapy plays an important role in local control, consolidation and palliative reduction of lymphoma.
Role of radiotherapy in lymphoma treatment
Radical treatment
For certain early stage lymphomas, such as early stage Hodgkin’s lymphoma without poor prognostic factors, early stage mucosa-associated tissue lymphoma, follicular lymphoma, etc., radiotherapy alone can achieve radical effect.
Consolidation of therapeutic effect
Reduce symptoms
For patients with poor tolerance to chemotherapy, especially those who have received too many courses of chemotherapy in the past or elderly patients, radiotherapy can reduce local symptoms, slow down disease progression, prolong survival time and improve quality of life.
Rescue treatment
For the spinal cord compression and gastrointestinal obstruction caused by certain lymphoma, local radiotherapy can rapidly release or reduce the compression, relieve the symptoms and finally achieve the effect of relief treatment.
Side effects of radiotherapy
Hematopoietic stem cell transplantation (HSCT)
Hematopoietic stem cell transplantation is the intravenous infusion of normal human hematopoietic stem cells into patients who have undergone pretreatment (chemotherapy/radiotherapy) to rebuild the patient’s hematopoietic and immune functions for the purpose of treating certain diseases.
The classification of HSCT can be divided into allogeneic HSCT (allo-HSCT) and autologous HSCT (auto-HSCT) according to donor genetics.
Allogeneic HSCT (allo-HSCT)
In the following cases, doctors may attempt autologous or allogeneic HSCT after high-dose combination chemotherapy with the aim of maximizing tumor cell killing and achieving longer-term remission and disease-free survival.
Autologous hematopoietic stem cell transplantation (auto-HSCT)
When autologous hematopoietic stem cell transplantation is used for lymphoma treatment, there is less chance of contamination of the graft with lymphoma cells, faster recovery of hematopoietic function, and is indicated for patients with bone marrow involvement or who have undergone pelvic irradiation.
Surgical treatment
As a systemic malignant tumor of the blood system, surgical resection of lymphoma is mostly not used as a conventional treatment. Surgical intervention is still required in some special cases, mainly including:
Other treatments
Monoclonal antibodies
Interferon
Interferon has antiviral and antitumor activity and immunomodulatory effects. It also has a partial palliative effect on mycosis fungoides, etc.
Anti-Helicobacter pylori (Hp) drugs
Gastric MALT lymphoma is treated with anti-Hp therapy in some patients with improvement of symptoms and even disappearance of lymphoma.
CAR-T therapy
CAR-T cellular immunotherapy, i.e. chimeric antigen receptor T cell immunotherapy, is currently available in China, such as Akilensai.
Akilensai is used for the treatment of adult patients with relapsed or refractory large B-cell lymphoma after previous second-line or above systemic treatment, including diffuse large B-cell lymphoma (DLBCL) non-specific type (NOS), primary mediastinal large B-cell lymphoma (PMBCL), high-grade B-cell lymphoma, and follicular lymphoma-transformed DLBCL.
Prognosis
In general, Hodgkin’s lymphoma is slightly slower in progression, has a slightly longer course, responds better to treatment, and is even partially curable.
Non-Hodgkin’s lymphoma (except for the low-grade malignant type) tends to develop more rapidly, has a shorter disease course, has a variable response to treatment, and is prone to relapse even after remission, resulting in a poorer prognosis.
Survival rate
Hodgkin’s Lymphoma
Early-stage Hodgkin’s lymphoma has a better prognosis, for example, the 5-year survival rate of Stage I and Stage II is more than 90%.
Survival rates for advanced Hodgkin’s lymphoma need to be extrapolated based on the International Prognostic Score.
Non-Hodgkin’s Lymphoma
Non-Hodgkin’s lymphoma is a heterogeneous group of lymphomas, and the International Prognostic Index (IPI) is now commonly used as a prognostic stratification evaluation, with wide variations in survival rates, which need to be determined according to different risk groupings.
【Tips】For more on survival rates, please refer to reading the entries for each subtype of lymphoma.
Special Reminder.
The overall survival time of cancer patients can be roughly predicted by the 5-year survival rate, which refers to the proportion of patients whose tumors survive for more than 5 years after various comprehensive treatments.
The probability of recurrence after 5 years is very low and can generally be regarded as clinical cure.
Statistics such as the 5-year survival rate are only used for clinical research and do not represent the specific survival period of an individual. The expected survival time of an individual patient is affected by a variety of factors, so it is recommended to consult with the consulting physician.
Prognostic factors
The prognostic factors for different pathological types of lymphoma are different and complex. For more specific prognostic factors, please refer to the article on the various subtypes of lymphoma.
Daily
Life Management
Mindset and Mood
Living
Dietary regulation
Rest and Exercise
Review and Follow-up
Lymphoma patients should pay attention to self-monitoring of the condition, regular follow-up, and timely consultation if there is weakness, fever, night sweats, emaciation, and enlarged lymph nodes.
Lymphoma follow-up can refer to the recommended criteria of the 2014 Lugano Conference, please follow the doctor’s instructions.
Follow-up content
Medical history, physical examination, routine laboratory tests, imaging.
For patients with more than 1 year of follow-up, minimize CT or MRI and replace them with chest radiographs and ultrasound.
PET-CT is usually not recommended as a follow-up examination.
Frequency of follow-up
Curable types
Incurable types
Such as follicular lymphoma, set cell lymphoma, etc.
It is recommended to review every 3 to 6 months for the rest of your life.
Prevention
There is no standard prevention program for lymphoma, but paying attention to the following may help reduce the incidence of lymphoma.
Healthcare for children
The lymphatic system is related to the whole body, so it is important to guard against viruses in daily life.
During infancy, children should be immunized with various vaccines on a date-by-date basis, and from an early age, they should develop the habit of exercising to strengthen their bodies and build a line of defense against various viruses and bacteria.