Lymphoma is a malignant tumor originating from the lymphopoietic system. It mainly manifests as painless lymph node enlargement, hepatosplenomegaly, and all tissues and organs of the body can be involved, accompanied by systemic symptoms such as fever, night sweats, emaciation, and pruritus.
According to the tumor cells, there are two types of lymphoma: non-Hodgkin’s lymphoma (NHL) and Hodgkin’s lymphoma (HL). The pathological features of Hodgkin’s lymphoma are lymphocytes, eosinophils, plasma cells and specific Reed-Steinberg cells in the tumor tissue. HL is divided into nodular lymphocyte-rich type and classic type according to pathological types, the latter including lymphocyte-dominant type, nodular sclerosis type, mixed cell type and lymphocyte-ablative type.
The incidence of NHL is much higher than that of HL, and it is the sum of a group of independent diseases with strong heterogeneity. Pathologically, it is mainly lymphocytes, histiocytes or reticulocytes with different degrees of differentiation, and according to the natural course of NHL, it can be classified into three major clinical types, namely highly aggressive, aggressive and inert lymphomas. Based on the different lymphocyte origins, they can be classified as B-cell, T-cell and NK-cell lymphomas.
Etiology is unclear
It is generally believed that it may be related to genetic mutations, as well as viral and other pathogenic infections, radiation, chemical agents, combined autoimmune diseases, etc.
Clinical manifestations
Malignant lymphoma is a large group of tumors with considerable heterogeneity. Although it is usually found in the lymph nodes, the distribution characteristics of the lymphatic system make it a systemic disease that can invade almost any tissue and organ in the body. Therefore, the clinical manifestations of malignant lymphoma have certain common features, but at the same time, there are great differences according to different pathological types, invasion sites and scopes.
Local manifestations include superficial and deep lymph node enlargement, mostly painless, smooth and movable on the surface, tough, full and uniform on the palpation, active in the early stage, isolated or scattered in the neck, axilla, groin, etc., and fused with each other in the late stage, adhering to the skin, inactive, or forming ulcers; pharyngeal lymphatic ring lesions of the oropharynx, tongue, tonsils and nasopharynx have abundant lymphatic tissue in the mucosa and submucosa, forming pharyngeal lymphatic ring. The majority of lymphomas in the nasal cavity are NHL, and the main pathological types include nasal NK/T-cell lymphoma and diffuse large B-cell lymphoma; mediastinal lymph nodes in chest lesions are the most common sites of malignant lymphoma, mostly diffuse large B-cell lymphoma and precursor T-cell lymphoma in the mediastinum of HL and NHL.
On chest x-ray, there are round or round-like or lobulated shadows. The progression of the lesion may compress the bronchi and cause pulmonary atelectasis, and sometimes the central tumor necroses to form a cavity. Some lung lesions show diffuse mesenchymal changes, when clinical symptoms are obvious, often with cough, coughing, shortness of breath, dyspnea, and fever secondary to infection; malignant lymphoma may invade the myocardium and pericardium, showing pericardial effusion, and lymphoma invading the myocardium shows cardiomyopathy, which may have arrhythmia, abnormal ECG and other manifestations; abdominal manifestations spleen is the most common site of subdiaphragmatic invasion of HL.
The gastrointestinal tract is the most common site of extra-nodal lesions in NHL. Skin manifestations of malignant lymphoma can be primary or secondary skin invasion, mostly seen in NHL; bone marrow invasion of malignant lymphoma is manifested by bone marrow invasion or combined leukemia, which is one of the advanced manifestations of the disease, mostly in NHL; neurological manifestations: such as progressive multifocal leukoencephalopathy, subacute necrotizing myelopathy, sensory or motor peripheral neuropathy, and neuropathy. or motor peripheral neuropathy and other manifestations such as polymyopathy. Malignant lymphoma can also be primary or secondary to brain, epidural, testis, ovary, vagina, cervix, breast, thyroid, adrenal gland, retro-orbital tissues, larynx, bone and muscle soft tissues, etc. The clinical manifestations are complex and diverse, and should be differentiated.
Examination
1.Blood routine and blood smear
Blood routine is generally normal, but may be combined with chronic disease anemia; HL may show increased PLT, increased WBC and eosinophilia; aggressive NHL invading bone marrow may show anemia, decreased WBC and PLT, and peripheral blood may show lymphoma cells.
2.Bone marrow smear and biopsy
The lymphoma cells can be seen in the bone marrow smear with large cell size, rich chromatin, gray-blue color, and obvious abnormal morphology, and the “trailing phenomenon” can be seen; lymphoma cells ≥20% are considered as lymphoma leukemia; lymphoma cell aggregation infiltration can be seen in bone marrow biopsy. In some patients, increased phagocytosis and phagocytosis can be seen in bone marrow smear, mostly in T-cell NHL.
3.Blood biochemistry
LDH increase is related to tumor load, which is an indicator of poor prognosis; HL may have increased ESR and increased ALP.
4.Cerebrospinal fluid examination
Patients with moderately aggressive NHL clinical stage III/IV may have CNS involvement or CNS symptoms, cerebrospinal fluid examination is required, which shows increased pressure of cerebrospinal fluid, increased biochemical protein amount, increased number of conventional cells, mononuclear predominantly, and lymphoma cells can be found in pathological examination or flow cytometry examination.
5.Histopathological examination
The basic pathomorphological change of HL is the diagnostic R-S cells and their variant cells seen in a mixed proliferative background of multiple inflammatory cells. Immunohistochemical features: classic CD15+, CD30+, CD25+; nodal lymphocyte-dominant CD19+, CD20+, EMA+, CD15-, CD30-. NHL lymph node or histopathology is seen with destruction of normal lymph node or tissue structures and scattered or diffuse infiltration of tumor cells, with their own unique pathological manifestations and immunophenotypes depending on the pathological type.
Treatment
Lymphoma is highly heterogeneous and therefore treatment varies greatly. Lymphoma of different pathological types and stages differ greatly in terms of treatment intensity and prognosis. The treatment methods for lymphoma mainly consist of the following, but specific patients should be analyzed according to their actual conditions.
1.Radiotherapy
Certain types of lymphoma can be treated with radiotherapy alone in the early stage. Radiation therapy can also be used for consolidation therapy after chemotherapy and adjuvant therapy during transplantation.
2.Chemical drug treatment
Lymphoma chemotherapy is mostly combined with chemotherapy, which can be combined with targeted therapy drugs and biological agents. In recent years, the chemotherapy regimen for lymphoma has been greatly improved, and the long survival of many types of lymphoma has been greatly improved.
3.Bone marrow transplantation
Autologous hematopoietic stem cell transplantation can be considered for patients under 60 years of age who are moderate to high risk and can tolerate high dose chemotherapy. Some young patients with relapse or bone marrow invasion can also be considered for allogeneic HSCT.
4.Surgical treatment
Only limited to biopsy or complication management; combined with hypersplenism without contraindications, those with indications for spleen excision can have their spleen excised to improve the blood picture and create favorable conditions for future chemotherapy.