(1) Lymphadenopathy: More patients showed painless cervical lymphadenopathy in the early stage, and other parts were found in the later stage. Lymph nodes can be from soybean to jujube, with moderate hardness, toughness, uniformity and fullness. Generally, it does not stick to the skin, does not fuse in the early and middle stages, and can move. Advanced lymph nodes can grow very large, or they can fuse into large blocks with a diameter of more than 20cm. Some patients have multiple lymphadenopathy from the onset, and it is difficult to determine where the first site is.
(2) Mediastinum: Mediastinum is also one of the most common parts. Most patients often have no obvious symptoms at the initial stage. Invasive mediastinal lymph nodes can be single lymph node enlargement; It can also be that multiple lymph nodes are fused into a huge lump; The outer edge is wavy and invades one or both mediastinum, the latter is more common. Symptoms of oppression may appear later.
(3) Liver and spleen: Primary malignant lymphoma of the liver is rare, with only 1 case reported in the literature. Secondary liver invasion is not uncommon. The prognosis of patients with liver invasion is worse than that of patients with systemic symptoms.
(4) Extranodal organs: generally occur in NHL. In rare cases, HD may also have extra-nodal organs such as bone, pharyngeal lymphatic ring, skin, digestive tract and central nervous system.
2. Whole body performance
(65,438+0) Systemic symptoms: About 65,438+00% patients may take fever, itchy skin, night sweats and emaciation as the earliest clinical manifestations. With the development of the disease, fatigue and anemia appear. Generally, with the progress of the disease, systemic symptoms can be aggravated. Such patients may suffer from lymphopenia. Malignant lymphoma of mediastinum and retroperitoneum with fever and itchy skin.
Persistent fever, excessive sweating, weight loss, etc. It may indicate the progress of the disease and the failure of immune function, so the prognosis is not good. However, some patients only have itching and fever, and there is no huge lump. Those who get better quickly after treatment have a better prognosis.
(2) Skin damage: Patients with malignant lymphoma may have a series of nonspecific skin manifestations, and the incidence rate is about 13% ~ 53%. Common ones are pellagra papules, herpes zoster, systemic herpetic dermatitis, pigmentation, ichthyosis, exfoliative dermatitis and so on. Urticaria, erythema nodosum, dermatomyositis, acanthosis nigricans and pigmented urticaria can also occur. Scratches and skin infections caused by skin itching are more common. The immune status of patients with advanced malignant lymphoma is low, and skin infections often break and ooze for a long time, resulting in scattered skin thickening and desquamation.
(3) Anemia: About 10% ~ 20% of patients with malignant lymphoma have anemia at the time of treatment, even a few months before lymphadenopathy. Anemia is more common in advanced patients. Progressive anemia and accelerated erythrocyte sedimentation rate are important indexes to judge the development of malignant lymphoma in clinic.
(4) Nervous system manifestations: Patients with malignant lymphoma may have a series of nonspecific nervous system manifestations, such as progressive multifocal leukoencephalopathy, subacute necrotizing myelopathy, sensory or motor peripheral neuropathy and polymyopathy. The nature of the lesion can be as follows: ① degeneration; ② Demyelination; ③ infectivity; ④ Necrosis or mixed existence.
(5) Low immune function: Due to the low immune status of HD patients, especially those in the late stage, central nervous system infections, such as Cryptococcus neoformans, may occur; Hematogenous purulent meningitis or brain abscess may also occur. Malignant lymphoma invading brain parenchyma may be accompanied by cerebral hemorrhage.
The diagnosis of malignant lymphoma mainly depends on clinical manifestations, X-ray examination and pathological examination, but pathological examination is very important for the diagnosis and classification of malignant lymphoma.
Diagnostic treatment, clinically, we can often see that some patients have low fever due to long-term emaciation, fatigue or unknown reasons; Or in some cases, some people have swollen lymph nodes, and diagnostic radiotherapy is performed because they are worried about the spread caused by biopsy. However, a considerable number of patients were later proved not to be malignant lymphoma.
1. The diagnosis of standard lymphoma is based on pathological examination.
Reed-Sternberg cells are the characteristics of HL. R-S cells originated from B cells, with large volume, rich cytoplasm and shallow nuclear chromatin. They should have at least two nuclear lobules or nucleoli (called Hodgkin cells if they are mononuclear), and the immunophenotype is CD30 and CD 15 positive. According to other pathological features, HL is usually divided into four subtypes: nodular sclerosis type, mixed cell type, lymphocyte-dominated type and lymphocyte attenuation type; In WHO classification, another subtype, nodular lymphocytes, whose tumor cells are similar to popcorn cells, are variants of R-S cells.
The basic pathological features of NHL are: the normal structure of lymph nodes disappears and is replaced by tumor tissue; Proliferated lymphocytes are atypical; Tumor cells invaded the lymphatic sac. According to the morphological, immunological and molecular biological characteristics of tumor cells, NHL can be divided into many subtypes. At present, the classification methods widely used in the world are real classification and WHO classification, while China is accustomed to applying the "work plan" from 65438 to 0982 in the United States.
After the diagnosis of lymphoma, the disease should be staged according to AnnArbor standard.
2. The diagnosis and evaluation of lymphoma depends on pathological examination, and obtaining enough and suitable pathological specimens is the first condition for correct diagnosis. Usually accompanied by superficial lymphadenopathy, lymph node biopsy can be performed routinely. Lymph nodes in mediastinum or abdominal cavity are enlarged, but those who lack superficial lymph nodes need laparotomy or thoracotomy to obtain specimens. When deep lymph nodes fuse into huge masses, the puncture effect with Tru-Cut needle is also quite satisfactory. Only splenomegaly, when lymphoma is highly suspected in clinic, splenectomy should be done in time, and liver biopsy should be done during operation to obtain more diagnostic basis. When the liver is diseased, liver puncture can be performed under the guidance of CT or ultrasound to obtain the required liver tissue.
Gastroenteroscopy and microscopic biopsy are very important for the diagnosis of gastrointestinal lymphoma, but the pathological results of biopsy are not completely consistent with those after operation, and the non-conformity rate of a group of cases is 25.8%.
A few NHL showed fever, jaundice, abnormal liver function, decreased whole blood cells or neuromuscular symptoms in the early stage of the disease, and there was no definite contraindication to tumor mass or biopsy. At this time, bone marrow examination is very important. Bone marrow puncture and biopsy should be carried out at the same time, and repeated several times if necessary, and new techniques such as chromosome, immunophenotype and gene rearrangement should be tested as far as possible to make a definite diagnosis as soon as possible.
A biopsy failed to make a definite diagnosis, so lymphoma cannot be ruled out rashly. Of the 200 NHL patients, 13.2% were diagnosed by multiple biopsies. Therefore, it is recommended to consult a number of pathologists in the following cases.
(1) The pathological report of the biopsy specimen is inconsistent with the postoperative specimen.
(2) The pathological report of the other hospital is inconsistent with that of our hospital.
(3) The pathological reports of multiple biopsies are inconsistent.
(4) The pathological results are suspicious and inconsistent with the clinic.
Typical lymphoma is not difficult to diagnose. However, clinicians should pay enough attention to the scope and stages of the disease. When lymphoma is diagnosed by pathological examination, bone marrow examination, chest and abdomen CT and total gastrointestinal barium meal radiography should be done as much as possible. Although ultrasonic examination is cheap and easy, it has poor repeatability and lacks long-term preserved images. Only suitable for initial screening and follow-up after treatment.
The staging of lymphoma is an important basis for making treatment plans, especially in HL. At present, the internationally adopted AnnArbor staging standard (197 1 year, 1989 Cotswald revision) is mainly applicable to HL. For NHL, this staging standard can not predict the prognosis of the disease well, and can be used to make a rough staging. In the application of AnnArbor staging, a common problem is how to determine whether it is a localized lesion (stage ⅰ) or a diffuse lesion (stage ⅳ) when extranodal organs (or tissues) are involved, which is not described in detail in this literature. It can be understood that when the whole organ is enlarged and imaging can't distinguish a single lesion, it is a diffuse lesion.
Lymphoma is a heterogeneous disease. According to its pathological characteristics, it can be divided into two categories: HL and NHL, and each category has multiple subtypes. After half a century's efforts, pathologists all over the world have formulated a variety of classification standards, and 1994 has gradually unified into a real scheme. Based on the real scheme, WHO proposed the WHO classification in 2000. Based on the data provided by morphology, immunology and genetics, WHO classification emphasizes that each subtype may become an independent disease, and the determination of subtypes does not depend on the experience of individuals or groups, which should be widely recognized worldwide. WHO said that with the development of technology and the deepening of scholars' understanding of lymphoma, the WHO classification will be revised and improved continuously.
Some doctors once thought that the classification of lymphoma subtypes was too complicated and of little value for clinical treatment. However, more and more evidence shows that different subtypes of lymphoma may have special treatment methods. For example, if gastric MALT lymphoma is related to helicobacter pylori, antibiotic treatment is effective; Monoclonal antibodies are suitable for inert B-cell lymphoma. For ALK- anaplastic large cell lymphoma, autologous hematopoietic stem cell transplantation should be carried out as soon as possible. Therefore, pathologists and clinicians in China should learn and accept this classification and actively participate in its further revision.
Generally speaking, unless there are special indications (for example, some patients have large masses or long-term fever, and radiotherapy or chemotherapy are given several days before operation to create conditions for surgical resection), this diagnostic treatment is not suitable. The reasons are as follows: ① The existing radiotherapy and chemotherapy is not a specific therapy for malignant lymphoma, but also inhibits inflammation, tuberculosis, other granulomas and tumors. Therefore, in fact, these treatments can not be used to identify the nature of the disease. On the contrary, it makes the diagnosis more confusing because of covering up contradictions, and even sometimes biopsy can't make a clear diagnosis because of tissue necrosis, which brings difficulties to future treatment. ② Radiotherapy and most existing chemotherapy drugs have immunosuppressive effects, which will bring opposite effects to patients and promote the development of recessive infection; (3) Short-term and long-term effects of radiotherapy and chemotherapy (such as skin reaction, bone marrow suppression, and effects on children's bone development). ) are not good for patients.
Even patients with malignant lymphoma who have been diagnosed will sometimes have fever or individual lymph node enlargement during the observation period after treatment, which can not be attributed to "recurrence" without thinking, but should look for other possible reasons. Due to the influence of the disease itself and long-term treatment, such patients often have low immune function and are prone to catch a cold or systemic inflammation, so they are more likely to have fever or swollen lymph nodes in a certain part. If it is not handled properly, giving chemotherapy again will bring great harm to patients. We have reported (1978) a case of HD, which was well staged after treatment, but later continued to have fever and radial shadows in both lung hilus, and was ineffective after various anti-infection and antifungal treatments. Therefore, it is suspected that HD recurred and invaded the lungs, and chemotherapy was carried out, but it was later confirmed as tuberculosis by autopsy. No remaining HD found. Another young patient was admitted to hospital with progressive dyspnea, cyanosis and upper body edema. Chest X-ray showed a huge shadow in the middle mediastinum, and it was diagnosed as mediastinal malignant lymphoma with superior vena cava compression. Oxygen inhalation, hydrocortisone and nitrogen mustard were given immediately, and the patient was relieved obviously the next day and could move freely. After chest X-ray examination, the diagnosis result is the same as before. After primary chemotherapy, it was changed to radiotherapy, but the shadow did not continue to shrink after a little reduction. After discussion, thoracotomy was diagnosed as tuberculosis. These lessons can serve as a warning.