Introduction:
Hodgkin's disease(Hodgkin?s disease), also known as lymphoreticular sarcoma, is a chronic progressive, painless lymphoid tissue tumor whose primary tumor is mostly eccentrically distributed, originating from one or a group of lymph nodes, originating from the neck. Lymph nodes are more common, gradually spreading to adjacent lymph nodes, and then invading tissues such as the spleen, liver, bone marrow, and lungs. Due to the different sites of the disease, its clinical manifestations are diverse. There is very little morbidity before the age of 5, and gradually increases after the age of 5, the incidence of puberty is significantly increased, and the peak is 15 to 34 years old. There are more males than females. The ratio of male to female is 3:1 for 5 to 11 years old and 1.5:1 for 19 to 19 years old.
Cause:
In the United States, the number of newly diagnosed cases is 6000~7000. The ratio of male to female is 1.4:1.10 years old. The distribution of the two age peaks is 15~34 years old and 60 years old respectively. Because most cases are diagnosed after 60 years old. For non-Hodgkin's lymphoma (see below, and thus the second peak may be caused by human factors with inaccurate pathological diagnosis. Epidemiological studies have not found parallel evidence. The cause is unknown, but the patient seems to have genetic susceptibility Sensual and environmental relevance (eg loggers; EB virus infection; HIV infection).
The lymph nodes of the lesion were swollen and the normal structure was destroyed, and some or all of them were replaced by tumor tissues. Microscopically, lymph nodes are infiltrated, such as granuloma. Among them, mononuclear or multi-core Sternberg-Reed cells, lymphocytes, eosinophils and plasma cells are infiltrated, and fibrous tissue can be formed. Finding Si-Rui cells is the basis for diagnosing this disease.
In recent years, it has been found that different pathological changes have a great relationship with prognosis. In order to adopt different effective treatments, further classification is necessary. At present, Rye classification is adopted internationally and domestically, and it is divided into four types according to the prognosis.
1. lymphocyte dominant type;
2. Nodular sclerosis type;
3. Mixed type;
4. Lymphocyte reduction type.
symptom:
The clinical manifestations vary widely, mainly due to pathological classification, the location of the primary tumor and the affected organs, and the early or late stages of the disease. The earliest manifestations were that the superficial lymph nodes showed painless progressive enlargement, often lacking systemic symptoms, and progressed slowly. About 60% of the origin is in the cervical lymph nodes, which are less common in the supraclavicular, infraorbital and inguinal lymph nodes. At the beginning, the lymph nodes are soft and do not stick to each other, and there is no tenderness. It grows rapidly in the later stage and can stick to a huge mass. It is characterized by no inflammation in adjacent tissues and cannot be used to explain the cause of lymphadenopathy. A swollen lymph node can cause local compression symptoms, such as mediastinal lymphadenopathy that compresses the tracheobronchial, causing a dry cough. Abdominal pain without cause can be caused by enlarged peritoneal lymph nodes. Systemic symptoms can be low-heat, or characteristic regression heat, that is, after a few days of high fever, there can be a few days or weeks of Pel-Ebstein fever. There are often loss of appetite, nausea, night sweats and weight loss. These symptoms often do not occur when the lesion is limited. Itchy skin is a common symptom in adults. It is rare in children and does not occur even when a wide range of organs are invaded. About a quarter of children have metastasized to tissues other than lymph nodes at the time of diagnosis, and are more common in the spleen, liver, lung or bone and bone marrow. X-ray changes in the lung infiltration are mostly villous exudative changes, which are indistinguishable from fungal infections, with more rapid breathing and fever, and even respiratory failure. Liver involvement, intrahepatic biliary obstruction symptoms, moderate liver enlargement, scleral yellow staining, serum direct and indirect bilirubin and alkaline phosphatase increased. Bone marrow infiltration results in neutrophils, thrombocytopenia, and anemia. Mucosal ulcers and gastrointestinal bleeding can occur in the digestive tract. Lymphoma occurs in the epidural space of the spinal cord and can cause compression symptoms. In addition, various immune dysfunctions such as immune hemolysis, thrombocytopenia or nephrotic syndrome may occur.
Hodgkin's disease itself, or due to chemotherapy can cause low cellular immune function, such children are prone to secondary infections, about one-third of children with herpes zoster, and can spread to invade lung tissue. Fungal infections such as cryptococcus, corpus corpus, and Candida albicans are also common complications, and the lesions are more extensive.
For elderly children with persistent cervical lymphadenopathy, the disease should be suspected, because patients with this age group have a rare cervical lymphadenopathy due to inflammation of the upper respiratory tract. Chronic lymphadenopathy that cannot be found in other areas should also be thought of. The medical history should be detailed and a comprehensive physical examination should be performed. The final diagnosis depends on the pathological examination of the lymph nodes, and the larger lymph nodes should be taken for pathological examination. Puncture and absorption of lymphoid tissue is too unreliable because of too little material.
diagnosis:
The disease must be differentiated from chronic suppurative lymphadenitis, lymphatic tuberculosis, infectious mononucleosis, and lymph node metastasis of malignant tumors. Lymph node reactive hyperplasia caused by local chronic inflammation is sometimes difficult to distinguish from this disease.
complication:
Bone involvement can cause pain and vertebral osteoblastic lesions ("ivory" vertebrae); rare is osteolytic lesions with compression fractures.
3. The jaundice can occur due to lumps of the intrahepatic and extrahepatic bile ducts.
Pelvic or inguinal lymphatic obstruction can cause lower extremity edema.
Tracheal bronchial compression can cause severe dyspnea and wheezing. Infiltration of lung parenchyma can be similar to lobular sclerosis or bronchial pneumonia, and can cause cavities or lung abscesses.
treatment:
Chemotherapy or radiotherapy can cure most patients. The dose of 4000~4500cGy in the irradiation field within 4~4.5 weeks can cure lymphadenopathy in more than 95% of patients. In addition, standard therapy should include 3600cGy irradiation adjacent to the uninfringed area (enlarged Irradiation field, because the lesion is spread through the adjacent lymph. Although radiotherapy and chemotherapy are often recommended, patients with E subtype may also be effective for radiotherapy. For special cases, the first radiotherapy may be considered without staging, but the treatment plan Still based on staging.
Stage I and IIA diseases can be treated with radiation alone, but the treatment field should be enlarged, including the area where all lymph nodes are distributed on the palate. Most patients should expand to the lymph nodes and aortic bifurcation around the aorta as well as the spleen or spleen. This treatment can cure about 80% of patients. The cure refers to disease-free survival 5 years after treatment, and the recurrence is extremely rare. The large-scale invasion of the mediastinum (> 1/3 of the diameter of the thoracic cavity), the recurrence rate of single radiotherapy High, post-chemotherapy radiotherapy can prolong the recurrence-free survival of about 75% of patients. For patients with special IA, nodular sclerosis, or lymphocyte predominance, cloak-type irradiation alone may be sufficient.
Stage IIIA1 disease, irradiation of all lymph nodes (cloak type and inverted Y type) can achieve a total survival rate of 85% to 90%, and a 5-year disease-free survival rate of 65% to 75%. In some special cases ( If there is only mild spleen disease, a smaller range of radiation therapy (minus pelvic irradiation field) is equally effective. For IIB and IIIA1 diseases, radiotherapy and chemotherapy can be used, and combination chemotherapy is often used for stage IIIA2 disease. Radiation therapy with or without lymph nodes. The cure rate has reached 75% to 80%.
Because radiotherapy alone can not cure stage IIIB Hodgkin's disease, it is necessary to use combined chemotherapy or combined chemotherapy combined with radiotherapy, the survival rate is 70% to 80%.
Stage IV A and B diseases, using a combination chemotherapy regimen, especially with the MOPP regimen (nitrogen mustard,Vincristine,Methyl benzamidine, prednisone) or ABVD regimen (doxorubicin, bleomycin, vinblastine, azomethamine). According to recent randomized studies, ABVD has become the standard protocol for the treatment of most Hodgkin's patients. The program has resulted in complete remission in 70% to 80% of patients, and >50% persists in 10 to 15 years. The results of the prospective study did not demonstrate the efficacy of alternating MOPP with ABVD or other drug combinations. Better than ABVD. Other effective drugs are nitrosourea, streptozotocin, cisplatin and etoposide. Patients who fail to relapse or relapse within 6 to 12 months have a poor prognosis. The efficacy of rescue therapy is limited. The transplantation of autologous bone marrow or peripheral blood stem cells can be carried out in selected cases. For patients who are tolerant to the treatment of systemic conditions and effective in re-induction chemotherapy, the self-transplant cure rate can reach 50%. Allogeneic transplantation It seems to be inferior and therefore not recommended. Self-transplantation has been studied in patients with newly diagnosed Hodgkin's disease.
prevention:
Mainly aimed at possibleMalignant lymphomaVarious factors are prevented. It is currently believed that the loss of normal immune surveillance function, the tumorigenic effect of immunosuppressants, the activity of latent viruses and certain physical (such as radiation), chemical (such as anti-epilepsyThe long-term application of drugs, adrenocortical hormones, may lead to the proliferation of lymphatic network and eventually malignant lymphoma. Therefore, pay attention to personal and environmental hygiene, avoid drug abuse, and pay attention to personal protection when working in a harmful environment.
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