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.
The tumor cells are divided into two categories: 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, and HL is divided into nodular lymphocyte-rich type and classic type, which includes lymphocyte-dominant type, nodular sclerosis type, mixed cell type and lymphocyte-ablative type, It is the sum of a group of independent diseases with a strong heterogeneity, mainly lymphocytes, histiocytes or reticulocytes with different degrees of differentiation. 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.
The etiology 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 drugs, combined autoimmune diseases, etc.
Clinical manifestations of malignant lymphoma are a large group of tumors with considerable heterogeneity. Although they preferably occur in lymph nodes, the distribution characteristics of the lymphatic system make lymphoma a systemic disease, which can invade almost any tissue and organ of the body. Therefore, the clinical manifestations of malignant lymphoma have certain common features, and at the same time, there are great differences according to different pathological types, invasion sites and scopes.
Local manifestations: including superficial and deep lymph nodes enlargement, mostly painless, smooth and movable surface, tough, full and even texture, early activity, isolated or scattered in the neck, axilla, groin, etc., and late fusion, adhesion with skin, inactivity, or ulcer formation; pharyngeal lymphatic ring lesions oropharynx, tongue root, tonsils and nasopharynx have rich lymphatic tissue under the mucosa and mucosa, forming 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 thoracic lesions are a good site for malignant lymphoma, mostly diffuse large B-cell lymphoma and precursor T-cell lymphoma in HL and NHL in the primary mediastinum. lymphoma. The lesion may progress and cause bronchial atelectasis, sometimes with central necrosis and cavity formation. Some lung lesions show diffuse mesenchymal changes, and clinical symptoms are obvious at this time, often with cough, sputum, 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 and abnormal electrocardiogram; abdominal manifestations spleen is the most common subdiaphragmatic invasion site 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 to 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: progressive multifocal leukoencephalopathy, subacute necrotizing myelopathy, sensory or motor peripheral neuropathy, and neuropathy. Neurological manifestations: such as progressive multifocal leukoencephalopathy, subacute necrotizing myelopathy, sensory or motor peripheral neuropathy, and polymyopathy. Malignant lymphoma can also be primary or secondary to brain, epidural, testis, ovary, vagina, cervix, breast, thyroid, adrenal gland, retro-orbital tissue, larynx, bone and muscle soft tissue, etc. The clinical manifestations are complex and diverse, so attention should be paid to differentiation.
Systemic manifestations: including systemic symptoms such as fever, pruritus, night sweats and emaciation may appear before or at the same time as lymph node enlargement in malignant lymphoma. Immunological and hematological manifestations of malignant lymphoma may include anemia in 10%-20% of cases, and some patients may have increased white blood cell count and platelet count and increased sedimentation. The elevation of lactate dehydrogenase is related to tumor load. Some patients, especially in advanced stages, show abnormal immune function, and in B-cell NHL, monoclonal immunoglobulins can be detected in the serum of some patients in varying amounts.
Skin lesions Patients with malignant lymphoma may have a series of nonspecific skin manifestations with polymorphic skin damage, erythema, blistering, and erosions. Patients with advanced malignant lymphoma have a low immune status, and skin infections often break down and ooze over time, resulting in generalized scattered skin thickening and desquamation.
Blood tests and blood smears are usually normal, but may be combined with chronic disease anemia; HL may show increased PLT, increased WBC, and increased eosinophils; aggressive NHL invading bone marrow may show anemia, decreased WBC and PLT, and peripheral blood may show lymphoma cells.
Bone marrow smear and biopsy are rare, but if NHL invades the bone marrow, lymphoma cells can be seen in the bone marrow smear, which are large in size, rich in chromatin, gray-blue in color, with obvious morphological abnormalities and “trailing phenomenon”; lymphoma cells ≥20% are considered lymphoma leukemia; bone marrow biopsy shows lymphoma cell aggregation and infiltration. In some patients, increased phagocytosis and phagocytosis can be seen on bone marrow smear, mostly in T-cell NHL.
Increased blood biochemistry is associated with tumor load and is an indicator of poor prognosis. hL may have increased ESR and increased ALP.
Cerebrospinal fluid examination in patients with highly aggressive NHL clinical stage III/IV may show CNS involvement, or those with CNS symptoms need to have cerebrospinal fluid examination, which shows increased cerebrospinal fluid pressure, increased biochemical protein amount, increased conventional cell count, mononuclear predominant, and lymphoma cells can be found by pathological examination or flow cytometry.
The basic pathomorphological change on histopathological examination 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 type CD19+, CD20+, EMA+, CD15-, CD30-. NHL lymph node or histopathology is seen with disruption 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.
Diagnosis of lymphoma has a variety of clinical manifestations, although it can have chronic, progressive, painless lymph node enlargement, it can also manifest as other systemic involvement or systemic symptoms. When lymphoma is clinically suspected, pathological sections (biopsies) of lymph nodes or other involved tissues or organs may be performed to confirm the diagnosis.
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 main treatments for lymphoma are as follows, but the specific patient should be analyzed according to the actual situation of the patient.
Radiation therapy Certain types of lymphoma can be treated with radiation therapy alone in the early stages. Radiation therapy can also be used for consolidation therapy after chemotherapy and adjuvant therapy during transplantation.
Chemotherapy for lymphoma is mostly combined with chemotherapy, which can be combined with targeted therapeutic agents and biological agents. In recent years, chemotherapy regimens for lymphoma have been greatly improved, and long survival has been greatly improved for many types of lymphoma.
Bone marrow transplantation can be considered for autologous hematopoietic stem cell transplantation in patients under 60 years of age who are moderate to high risk and can tolerate high doses of chemotherapy. Allogeneic HSCT may also be considered in some young patients with relapse or bone marrow invasion.
Surgical treatment is limited to biopsy or management of complications; those with combined hypersplenism without contraindications and with indications for spleen excision can have their spleen removed to improve the blood picture and create favorable conditions for subsequent chemotherapy.
Prognosis
The prognosis of Hodgkin’s lymphoma is closely related to the tissue type and clinical stage. The lymphocyte-dominant type has the best prognosis with a 5-year survival rate of 94.3%, while the lymphocyte-depleted type has the worst prognosis with a 5-year survival rate of 27.4%. The 5-year survival rate of Hodgkin’s lymphoma is 92.5% for stage I, 86.3% for stage II, 69.5% for stage III, and 31.9% for stage IV. The prognosis is worse with systemic symptoms than without. The prognosis for children and the elderly is generally worse than that for young and middle-aged people; women are better than men after treatment.
The prognosis of non-Hodgkin’s lymphoma is as important as the type of pathology and stage. The 6-year survival rate is 61% for diffuse lymphocytes with good differentiation, 42% for diffuse lymphocytes with poor differentiation, and only 30% for lymphoblastoid lymphoma with 4-year survival rate. The presence or absence of systemic symptoms had less impact on prognosis than HL. The course of non-Hodgkin’s lymphoma in the low malignancy group is relatively mild, but there is a lack of effective cure, so it has a chronic course with multiple relapses, and there are also deaths due to transformation to other types and resistance to chemotherapy. However, if the low-grade malignant group is detected early, it can survive for 5 to 10 years or even longer with reasonable treatment. Some highly malignant lymphomas are sensitive to radiotherapy and their survival can be significantly prolonged with reasonable treatment.