What is Hodgkin's Disease?

Hodgkin's lymphoma (HL) is a unique type of lymphoma and is one of the most common malignancies in young people. The disease first occurred in a group of lymph nodes, which were common in cervical lymph nodes and supraclavicular lymph nodes, and then spread to other lymph nodes. In later stages, they can invade blood vessels and involve the spleen, liver, bone marrow, and digestive tract. Classical Hodgkin's lymphoma can be divided into 4 histological types: lymphocytic type, nodular sclerosis type, mixed cell type, and lymphocyte depletion type. In recent years, a type of nodular lymphocyte has been added to the WHO classification. The most common type in China is mixed cell type. Various types can be converted between each other. Histological subtype is the main factor that determines the clinical manifestations, prognosis and treatment of patients.

Basic Information

nickname

Hodgkin 's disease

English name

Hodgkin's lymphoma

Visiting department

Hematology

Multiple groups

Young men

Common locations

Cervical lymph nodes, supraclavicular lymph nodes

Common causes

unknown

Common symptoms

Painless lymphadenopathy with fever, night sweats, weight loss, etc.

Contagious

no

Causes of Hodgkin's lymphoma

The cause of Hodgkin's lymphoma is unknown, and EB virus genome fragments can be detected in RS cells in about 50% of patients. Patients with known immunodeficiency and autoimmune diseases are at increased risk for Hodgkin lymphoma.

Hodgkin's lymphoma clinical manifestations

Lymphadenopathy

90% of patients are treated with lymphadenopathy, most of which are cervical lymphadenopathy and mediastinal lymphadenopathy. Lymphadenopathy is often painless and progressive. Pain after drinking is a relatively specific manifestation of lymphoma diagnosis.

2. Extranodal lesions

Involvement of extra-lymph nodes in the late stages can cause anatomy and dysfunction of the corresponding organs, causing a variety of clinical manifestations.

3. Systemic symptoms

20% to 30% of patients present with fever, night sweats, and weight loss. Fever can be low fever, sometimes intermittent high fever. In addition, itching, fatigue and so on.

4. Clinical manifestations of different histological types

Nodular lymphocytic type (NLPHL) is more common in males and the male to female ratio is 3: 1. Lesions usually involve peripheral lymph nodes, and most are early localized lesions at the time of initial diagnosis. About 80% of them are stage or . The natural course is slow and the prognosis is good. The complete response rate can reach 90%, and the 10-year survival rate is about 90%. However, the prognosis of patients with advanced stage (, ) is poor. In classic Hodgkin's lymphoma, the lymphocyte-rich type accounts for about 6%, with an average age and more common in men. The clinical features are between nodular lymphocytic predominant and classic Hodgkin's lymphoma. They often show early localized lesions and have a good prognosis, but the survival rate is lower than NLPHL. Nodular sclerosis is the most common in developed countries. Common, more common in young adults and adolescents, slightly more women. It often manifests as lymph node lesions in the mediastinum and other parts of the condyle, with a good prognosis; mixed cell types account for 15% to 30% in European and American countries, and can occur at different ages. Clinical manifestations: Abdominal lymph nodes and spleen lesions are common. About half of the patients are at the advanced stage (stages III and IV) at the time of consultation, and the prognosis is poor. Lymphocytic depletion is rare, about 1%. It is more common in the elderly and people infected with human immunodeficiency virus (HIV). It often involves abdominal lymph nodes, spleen, liver, and bone marrow, often with systemic symptoms. The disease progresses rapidly and the prognosis is poor.

Hodgkin's lymphoma test

Laboratory inspection

Anemia is more common in advanced patients, and it is positive pigment and positive cell anemia. Occasionally hemolytic anemia, the Coombs test was positive in 2% to 10% of patients. In a few cases, neutropenia and eosinophilia may occur. Peripheral blood lymphocyte decrease (<1.0 × 10 ⁹ / L), rapid erythrocyte sedimentation, and elevated serum lactate dehydrogenase can be used as indicators for disease detection.

2. Histopathology

The normal lymphoid tissue structure such as the lymph nodes at the lesion is completely or partially destroyed, showing a variety of non-tumor reactive cell components, mostly lymphocytes, and plasma cells, eosinophils, neutrophils, tissue cells, and fibroblasts can be seen And fibrous tissue. There are a variety of typical RS cells and their variants scattered in various reactive cell background components. Typical RS cells are dinuclear or multinucleated giant cells with eosinophilic nucleoli, large and distinct, and abundant cytoplasm. If the cells show symmetrical dual nuclei, they are called "mirror cells." RS cells and atypical (variant) RS cells are considered to be true tumor cells of Hodgkin's lymphoma. Recently, single-cell microscopy combined with immunophenotypic and genotypic tests has proven that RS cells are derived from lymphocytes, mainly from B lymphocytes. Classical Hodgkin's lymphoma has positive CD15 and CD30 antigen expression in RS cells, which is an important immune marker for identifying RS cells.

3. Imaging diagnosis

(1) X-ray plain film can usually see asymmetric nodules in the bilateral anterior and superior mediastinum, and rarely show calcification, except after radiotherapy.

(2) CT can show multiple, large soft tissue masses with no necrosis, hemorrhage, or cystic changes, and enhanced scan enhancement is not obvious. Swollen nodules can eventually lead to significant space effects.

(3) MRI can show low T ₁ WI signal and uniform signal mass with high T ₂ WI signal intensity due to edema and inflammation.

(4) PET positron emission tomography (PET) is helpful for comprehensive evaluation of disease staging and treatment effect, and is currently used as an important imaging method.

Hodgkin's lymphoma differential diagnosis

The disease needs to be distinguished from diseases such as lymphatic tuberculosis, viral infections such as infectious mononucleosis, and non-Hodgkin's lymphoma, and attention should be paid to distinguishing from metastatic cancer. Neck enlargement should exclude nasopharyngeal cancer and thyroid cancer. Mediastinal masses should exclude lung cancer and thymoma. Axillary lymphadenopathy should be distinguished from breast cancer. The identification of the above diseases mainly depends on histopathological examination. Histopathological diagnosis is the necessary basis for the diagnosis of Hodgkin's lymphoma. Pathological diagnosis usually requires typical RS cells, combined with the overall tissue performance of lymphocytes, plasma cells, eosinophils and other reactive cell component backgrounds, and combined with immune markers such as CD15 and CD30 to make a diagnosis.

Hodgkin's lymphoma treatment

The application of modern radiotherapy and chemotherapy has made Hodgkin's lymphoma a curable tumor. However, the follow-up results of a large number of long-term survival patients show that the 15-year mortality rate is 31% higher than that of the general population. The cause of death is in addition to the recurrence Second tumors accounted for 11% to 38% (solid tumors and acute non-lymphocytic leukemia), 13% of acute myocardial infarction, and 1% to 6% of pulmonary fibrosis. In addition, radiotherapy and chemotherapy can cause infertility and deformities. These are the results of overtreatment. Therefore, for HL that can be cured, efficacy and quality of life are also issues of concern. This balance needs to be drawn from the results of a large number of prospective randomized controlled studies. Therefore, through the understanding of the complications of long-term treatment of HL, a new treatment strategy for preventing and reducing long-term serious complications and improving the quality of life was proposed. At present, the treatment plan of HL is mainly based on clinical stage and prognostic factors.

Radiation therapy alone

At present, it is believed that radiotherapy alone is only suitable for patients with stage IA NLPHL. For other patients, radiotherapy is only used as an adjuvant treatment for chemotherapy. Large-dose and large-scale radiotherapy brings a variety of long-term complications, so it is not recommended as a radical method.

2. Early stage (CS, ) HL with good prognosis

ABVD chemotherapy 2 to 4 courses plus 20-30 Gy of radiotherapy in the affected field.

3. Early (CS, ) HL with poor prognosis

ABVD chemotherapy 4 ~ 6 courses plus affected field or area 20 ~ 36Gy radiotherapy.

4. Late HL

6 to 8 courses of ABVD chemotherapy, accompanied by a large block plus 30 to 36 Gy of radiotherapy in the affected area or area.

5. Intractable or relapsed cases

A new regimen that does not have cross-resistance to the original regimen, such as ICE, DHAP, ESHAP, mini-BEAM, GDP, and ABVD / MOPP (or COPP) alternative regimens, should be used for treatment. High-dose chemotherapy can be selected after better remission Combined autologous hematopoietic stem cell transplantation.

6. Prevention of complications

In particular, the prevention and treatment of opportunistic infections in the immunosuppressive phase, such as tuberculosis, fungal infection, hepatitis and cytomegalovirus infection.

Hodgkin's lymphoma prevention

The cause of Hodgkin's lymphoma is unknown, so there is no conclusive evidence to prevent its occurrence. However, the following measures may be beneficial:

1. Prevent viral infections, such as EB virus, adult T lymphocyte virus, HIV, etc. Prevent colds in spring and autumn, strengthen self-protection, and overcome bad living habits.

2. Remove environmental factors, such as avoiding exposure to various rays and some radioactive substances, and avoid contacting related toxic substances, such as benzene, vinyl chloride, rubber, arsenic, gasoline, organic solvent coatings, etc.

3. Prevention and treatment of autoimmune deficiency diseases, such as immunocompromised status after various organ transplants, autoimmune deficiency diseases, and various cancer chemotherapy. These conditions can activate a variety of viruses, which can induce abnormal proliferation of lymphoid tissues and eventually lead to lymphoma.

4. Maintain an optimistic and confident healthy mentality and proper physical exercise will help stabilize the body's immune function and maintain the ability to monitor tumor immunity.

Hodgkin's lymphoma prognosis

The following factors have prognostic value at the initial diagnosis of Hodgkin's disease:

1. Clinical stage of disease: The earlier the stage of disease, the better the prognosis.

2. Histological subtype: The prognosis of lymphocyte predominant type and nodular sclerosis type is better than that of mixed cell type. The prognosis of lymphocyte depletion type is the worst.

3. Poor tumor cell load.

4. Poor people with systemic symptoms.

5. Those who are> 45 years old are worse.

6. The number of disease sites, the number of extranodal lesions, and the presence or absence of bone marrow lesions.

7. Gender: Women have slower disease progression than men.

8. Hemoglobin L, white blood cells> 15 × 109 / L, lymphocytes <0.6 × 109 / L.