Introduction:
He Jiejin disease(hodgkin's disease), also known as lymphoreticular sarcoma, is a chronic progressive, painless lymphoid tissue tumor. The primary tumors are mostly eccentrically distributed, originating from one or a group of lymph nodes, and originating from the cervical lymph nodes. More common, gradually spread to adjacent lymph nodes, and then invade 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:
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 is the best type of differentiation, can also be considered as the early stage of Hodgkin's disease, its degree of malignancy is relatively low, the lesion is often limited to one or a group of lymph nodes. The clinical symptoms are very mild or have no discomfort. Microscopically, lymphocytes and histiocytes showed different proportions of hyperplasia in the area where the normal lymphoid tissue disappeared, and the small lymphocyte proliferation was common; Si-Rui cells were rare and atypical. There is no necrotic change in the lymph nodes. This lymph node is the most difficult to distinguish from inflammatory lesions and is easily missed. This type accounts for about 10% to 20% of the disease and has the best prognosis.
2, nodular hardening type This type rarely evolved into other types. Occurs in the mediastinal lymph nodes, but also can affect the supraclavicular lymph nodes, rarely seen in the abdominal lymph nodes, clinical development is slow. There are more collagen fiber bundles in the lesion that divide the tumor cells into individual nodules. Si-Rui cells are commonly found in fissure-like blanks, also known as lacunar cells, which are the most common type in childhood, accounting for about half of the disease, and the prognosis is second only to lymphocyte dominant.
3, mixed type can be derived from the dominant type of lymphocytes. Most of the clinical symptoms have obvious symptoms. The structure of the lymph nodes disappears diffusely, but only a part of the structure is destroyed when the lymph nodes are initially involved. There are various cells in the lesion including lymphocytes, histiocytes, eosinophils and plasma cells, and there are typical binuclear and lobulated nuclei. Or multi-nuclear S-Rui cells with larger nucleoli. This type varies widely, and is typically easy to diagnose. Atypical is confused with inflammatory granuloma, tuberculosis, and reactive hyperplasia. This type accounts for more than 10% of the disease. There are many extranodal lymph nodes in the diagnosis, and the prognosis is poor.
4, lymphocyte reduction type can be directly evolved by lymphocyte dominant type, or converted from mixed type, is the late stage of lymphoma, is the worst type of differentiation, and the disease develops rapidly. There are few lymphocytes in the lesion, which can be divided into two forms, a diffuse sclerosis composed of transparent collagen fibers, lymphocytes are significantly rare, and the lymph node volume can be reduced. One is mainly composed of a large number of heterogeneous reticulocytes. - Wright cells are easy to find. This type accounts for about 10% and has the worst prognosis.
In children, there are more nodular sclerosis and lymphocyte predominance, which is the reason for the long survival period of Hodgkin's disease in children.
Staging is helpful in judging the prognosis and the choice of treatment options.
Stage I lesions are limited to one lymph node, or one anatomical region of the lymph node (I), or only one extranodal tissue has lesions (IE).
Stage II lesions are confined to two or more lymph nodes adjacent to the anatomical region, or two non-adjacent lymph nodes (II) on the ipsilateral side, or one side of the lymph nodes or the same side or several Lymph nodes have lesions (IIE).
Stage III lesions are on both sides of the transverse iliac crest (III), or at the same time invading the extranodal tissue (IIIE), with spleen invasion (IIIS) or both (IIIES).
Stage IV lesions extensively invade lymph node tissues, such as bone marrow, liver, lung, pleura, bone, skin, kidney, gastrointestinal tract, etc. with or without lymphadenopathy.
Each of the above issues is divided into two groups, A and B. Patients in group A had no systemic symptoms, and patients in group B had fever, night sweats, and no more than 10% weight loss within six months.
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 have low fever, or a characteristic regression heat type, which can be a few days or weeks of Pel-Ebstein fever after a few days of high 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.
He Jiejin disease itself, or due to chemotherapy can cause low cellular immune function, such children are prone to secondary infections, about one-third of the children with herpes zoster, and can spread the lung tissue. Fungal infections such as cryptococcus, corpus corpus, and Candida albicans are also common complications, and the lesions are more extensive.
diagnosis:
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.
After the diagnosis, the stage should be further staged. Since many patients have only one group of cervical lymphadenopathy after general examination, the diagnosis is stage I, but there is often a mediastinal or abdominal cavity metastasis. Therefore, only about one-third of the clinical examinations are inaccurate. Therefore, the following checks should be made.
(1) X-ray tomography of the chest and mediastinum: hilar lymphadenopathy and pulmonary infiltration can be found.
(2) inferior vena cava angiography and intravenous pyelography: the former can be found in the second lumbar vertebrae above the aortic lymph nodes, the latter can show whether the ureter is displaced, in addition to the service department radiotherapy needs to understand the location of the kidney.
(3) Pediatric lymphography: early detection of abdominal and aortic lymphadenopathy.
(4) Skeleton X-ray film to understand whether the bone is invaded.
(5) Determination of serum alkaline phosphatase: If the increase indicates the possibility of bone and liver metastasis.
(6) It is necessary to do liver and spleen scanning and liver function measurement: Ultrasound scan (B ultrasound scan) is very helpful for finding abdominal cavity lesions. If there is a high degree of suspicion that the abdominal lymph nodes have lesions, laparotomy can be performed, and spleen resection should be performed. The abdominal and posterior abdominal lymph nodes and liver tissues should be taken for pathological biopsy.
Identification
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:
A swollen lymph node can cause local compression symptoms, such as mediastinal lymphadenopathy that compresses the tracheobronchial, causing a dry cough.
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 appearsNeutrophil, 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.
treatment:
Western medicine treatment
In recent years, due to the combination of pathological typing, clinical staging and radiotherapy, chemotherapy, surgical treatment, etc., the efficacy has been significantly improved. Early diagnosis and treatment may be cured.
According to the principles of treatment proposed by Kaplan et al, the treatment can be as follows in five stages.
1. Stage I high-grade cervical lymph nodes, mediastinum and inguinal lymph node lesions were treated with local radiotherapy. Dosage and 30 to 40 Gy in 3 to 4 weeks can control the local mass of 80% of the sick children. The general dose is 35 Gy, and only a few cases need to be increased to 40 Gy.
2, stage II mediastinum and para-aortic lymph are also treated with radiotherapy, the dose is preferably no more than 30Gy, because the heart and spine are poorly tolerated by radiotherapy.
3, other types or parts of I and II in addition to local radiation therapy plus chemotherapy.
4, stage III is mainly chemotherapy, plus radiotherapy.
5, stage IV is mainly chemotherapy, and radiotherapy is added to the huge tumor block.
For stage I lesions, surgery can also be used, followed by radiation therapy.
In the childhood of rapid growth and development, the side effects of treatment must be carefully considered. Because radiation therapy can affect bone and soft tissue development, and even affect growth, in children under 8 years of age, as little as possible with radiotherapy to replace surgery and chemotherapy. Splenectomy should be postponed until after 5 years of age.
For patients with stage I, II, or stage I, II, and stage III, which are unreasonably poorly pathologically differentiated, chemotherapy and local radiotherapy should be used alternately, such as starting two courses of chemotherapy. Then use radiation therapy, and then use chemotherapy for a course of treatment.
The effective regimen commonly used in chemotherapy for children is as follows.
1, MOPP program is the most commonly used program in childhood, using nitrogen mustard (mustargen),Vincristine(oncovin),Methyl benzamidine(procarbazine) and prednisone four drugs combined treatment for 14 days, rest for 14 days for a course of treatment, sharing 6 courses. Children over the age of 8 or under the age of 8 years, the tumor site does not affect the growth and development, after two courses, start radiotherapy, and then undergo a course of chemotherapy. Can also be usedCyclophosphamide300 mg/m2 was used instead of ammonia mustard (COPP), and methotrexate was used instead of procarbazine.
2. Vincristine in the MVPP regimen was replaced with vinblastine 6 mg/m2.
The above two regimens have fewer side effects, and the myelosuppression phenomenon recovers faster during the withdrawal period of each course of treatment. For those who have not received chemotherapy, or who have relapsed with radiation therapy alone, about 80% of the patients who received the above regimen achieved complete remission after 6 courses of treatment.
If the effect of the above scheme is not significant, try other combination therapy, such as adriamycin, bleomycin, dimethyltriazeno-imidazole-carboxamide (DTIC) and chloroacetate. Chloroethyl-cyclohexyl-nitrosureas (CCNU) and the like. Can be selected according to the disease period, bone marrow tolerance, etc. Bleomycin has not been widely used in children with Hodgkin's disease.
Generally, after 6 courses of treatment are completed, the treatment can be relieved. At this time, the treatment should be continued. The interval of the original program should be repeated, such as repeating one course every 2 months in the first year and repeating one course every 3 months in the second year. In the third half of the third year, repeat a course of treatment. There are different opinions on whether there is a need to maintain treatment. Some people think that after 6 or 12 courses of treatment with MOPP, there is no difference in the time of remission between the withdrawal and the maintenance treatment.
For patients who have relapsed after radiotherapy, if the bone marrow can be tolerated, MOPP or other regimens can be used for treatment. If only local lymph node recurrence or extranodal infiltration occurs, but the patient is unable to tolerate chemotherapy, then local Radiation Therapy.
Radiation therapy combined with chemotherapy has a large side effect. Due to the application of immunosuppressants, the body's resistance is low, and it is easy to combine with viruses, fungi and protozoal infections. Injectable supportive therapy is needed, if necessary, transfusion or antibiotic treatment.
Due to the prolonged treatment time, attention should be paid to the treatment of advanced secondary disease. Radiation therapy should be applied during the rapid development stage (< years old or 12 to 13 years old), which may lead to slower growth and upper radiotherapy may affect sitting height. Neck irradiation can cause neck growth to stagnate; mediastinal and chest irradiation can cause breast developmental disorders, pleural fibrosis, pulmonary function limitation and cardiac involvement. Late treatment can cause hypothyroidism, and cases of esophageal stricture have also been reported. Where high-dose radiation therapy plus chemotherapy is used, there is a possibility of secondary malignancy, especially in patients who are relapsed after treatment. Acute non-lymphocytic leukemia is most common in secondary malignancies.
prevention:
He Jiejin's disease has been treated effectively and is no longer incurable. Foreign literature reports that 80% of patients with stage I and II have a survival of more than 5 years. The recurrence within 10 years is nearly 50%. At present, the remission time of IIIA has caught up with stage I and II, but the 5-year remission rate of stage IV patients is only 20%. Adult patients often have infertility after applying MOPP. Although there is not enough data for prepubertal children, it is speculated that it may have a certain impact on normal development.
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