Introduction:
Castleman disease(Castleman's disease, CD) is one of the unexplained reactive lymphadenopathy, clinically rare, characterized by significant swelling of deep or superficial lymph nodes, some cases may be associated with systemic symptoms and / or multiple systemic damage, most cases of surgery After removing the enlarged lymph nodes, the effect is good.
Cause:
(1) Causes of the disease
The cause of CD is unknown. Plasma cell type is thought to be related to infection and inflammation. Some authors suggest that immunomodulatory abnormalities are the initiating factors of CD. Clinically, 25% of central cases are confirmed to be associated with HHV-8 infection, and at least part of CD is considered to be in B cell malignant hyperplasia. Among the dangers, a few multi-center types can be converted into malignant lymphoma, but most cases follow the results did not translate into malignant tumors.
(two) pathogenesis
Because there are more than one vascular germinal center in the lymph node follicles, some cases also have angiolipoma components, and the lesions can also occur in the normal absence of lymphoid tissue, so it was considered a hamartoma, angiography The image is also similar to other vascular hamartomas. CDs dominated by plasma cell proliferation are thought to be associated with infection (mainly viral infection) and inflammation, due to pathologically inflammatory changes such as plasma cells, immunoblasts, and capillary proliferation, while retaining residual lymph node structures. Clinical signs of inflammatory lesions, such as chronic anemia, increased erythrocyte sedimentation rate, hypoalbuminemia, and increased polyclonal immunoglobulin. Some authors have suggested that immunoregulatory abnormalities are the initiating factors of CD. For example, the typical immunodeficiency disease, AIDS, can occur simultaneously with CD and Kaposi sarcoma, and a small number of CD can also be converted into Kaposi's sarcoma; clinical part The patient was associated with autoimmune cytopenia, antinuclear antibody positive, rheumatoid factor positive or anti-human globulin test positive; some immunological tests showed that some CD patients had antigenic responsiveness and T suppressor cells were absent. Some authors believe that CD is a pre-neoplastic lesion, because the immunohistochemical staining of plasma cells in CD lesions is monoclonal, individual immunoglobulins appear in the blood of individual patients, and a few multi-center patients can be converted into malignant lymphoma.
It has been reported that IL-6 is involved in the pathogenesis of CD. For example, IL-6 gene is transferred into hematopoietic stem cells of mice, and a pathological model similar to CD can be successfully obtained. It has also been confirmed that B lymphocytes of the lymph node germinal center of CD can secrete a large amount of IL-6. After the lesion is removed, as the clinical condition improves, the elevated serum IL-6 level also decreases. In addition, it was confirmed in animal experiments that human herpesvirus 8 (HHV-8), Kaposi's sarcoma herpesvirus (KSHV), is involved in the pathogenesis of CD.
symptom:
CD is clinically divided into focal type and multi-center type.
1. Focal young people are more common, the median age of onset is 20 years old. 90% of the pathology is transparent vascular type. The patient has a single lymph node that is painless and swollen, grows slowly, and forms a huge mass. The diameter is from a few centimeters to 20 cm. It can occur in any part of the lymphoid tissue, but the mediastinal lymph nodes are the most common, followed by the neck, ankle and abdominal lymph nodes. . Occasionally, extranodal tissues such as the larynx, vulva, pericardium, intracranial, subcutaneous, muscle, lung, eyelid, etc. have been reported. Most of them have no systemic symptoms, and the tumor can survive for a long time after resection, which is a benign course. 10% of the pathology is plasma cell type, abdominal lymph node involvement is more common, often accompanied by systemic symptoms, such as long-term low fever or high fever, fatigue, weight loss, anemia, etc., after surgery, the symptoms can all subside, and no recurrence.
2. The multi-center type is rare compared with the focal type. The age of onset is lower, and the median age is 57 years. The patient has multiple lymph nodes and easily spreads to superficial lymph nodes. With systemic symptoms (such as fever) and hepatosplenomegaly, often with multiple systemic manifestations, such as nephrotic syndrome, amyloidosis,Myasthenia gravis, peripheral neuropathy, temporal arteritis, Sjogren's syndrome (sjogren's syndrome), thrombotic thrombocytopenic purpura and oral, keratitis. 20% to 30% of patients may have Kaposi's sarcoma or B-cell lymphoma in the course of the disease. A small number of patients with multiple neuropathy, organ enlargement (liver, spleen), endocrine lesions, serum monoclonal immunoglobulin and skin lesions constitute a clinical sign of POEMS syndrome. In addition, multi-center clinical is often invasive disease, easy to be associated with infection.
The clinical manifestations of CD are non-specific. If the lymph nodes are obviously swollen, with or without systemic symptoms, the possibility of CD should be considered. The lymph node biopsy can be diagnosed by the typical CD pathological changes mentioned above, that is, the diagnosis of CD must have pathological evidence. Then according to the clinical manifestations and pathology, the classification diagnosis is made. It is also necessary to rule out various possible related diseases before diagnosis.
diagnosis:
CD should beMalignant lymphoma, a variety of lymph node reactive hyperplasia (mostly caused by viral infection), plasma cell tumor, AIDS and rheumatic diseases. They have some similar clinical manifestations and/or pathological changes, careful pathology, including immunohistochemistry, and detection of certain primary diseases are the primary points of identification. The lymph nodes of this disease must be differentiated from the following diseases:
1. Although lymphoma is swollen in both cases, the performance is different. Lymphoma may have persistent or periodic fever, general itching, splenomegaly, and weight loss. The clinical symptoms of this disease are mild, only the symptoms caused by fatigue or organ pressure, the most important is the pathological difference, the histological features of this disease is significant vascular proliferation.
2. Angioimmunoblastic lymphadenopathy is an abnormal non-neoplastic immunoproliferative disorder. Clinically more common in women, manifested as fever, systemic lymphadenopathy, may have rash and itchy skin, auxiliary examination of white blood cells increased, ESR increased, antibiotic treatment is ineffective, hormones can improve symptoms. Lymph node pathology is lymph node destruction, capillary wall hyperplasia, is an immune mother cell. PAS-positive vascular endothelial cells, amorphous substance deposition, and eosinophilic non-structural deposition between cells. Biopsy can be identified.
3. Primary macroglobulinemia The disease is mainly caused by lymphoid plasma cell proliferation and secretion of a large number of monoclonal macroglobulin, and extensive infiltration of bone marrow and extramedullary organs. A large number of individual IgM appeared in the serum, no bone destruction, no kidney damage, clinically liver, spleen, lymph node enlargement, about half of the patients with hyperviscosity.
4.Multiple myeloma It is a common type of plasma cell disease. Plasma cells (or myeloma cells) proliferating in the bone marrow infiltrate bones and soft tissues, causing a series of organ dysfunction, clinical manifestations of bone pain, anemia, renal dysfunction and immune dysfunction, hypercalcemia. Myeloma cells infiltrate the liver and spleen, lymph nodes and kidneys. CD lymph nodes are obvious, and lymph node biopsy can be identified.
complication:
1. About one-third of patients can have Kaposi's sarcoma or B-cell lymphoma.
2. Combined with nervous system, endocrine and renal lesions, Sjogren's syndrome (sjogren's syndrome), thrombotic thrombocytopenic purpura, etc.
treatment:
(a) treatment
Focal CD should be surgically removed, and most patients can survive for a long time, with fewer recurrences. Pathologically, the focal CD of the plasma cell type, if accompanied by systemic symptoms, can also disappear rapidly after the lymph node resection of the lesion.
Multi-center CD, such as lesions only invade a few parts, can also be surgically removed, plus chemotherapy or radiotherapy after surgery. The multi-center CD with extensive lesions can only choose chemotherapy, or the main lesions plus local radiotherapy, most of which can only be partially relieved. Chemotherapy is usually used in combination with chemotherapy for malignant lymphoma. Autologous hematopoietic stem cell transplantation is also a treatment option.
(two) prognosis
The disease is a focal lesion with a good prognosis, and the multi-centered with monoclonal hypergammaglobulinemia has a poor prognosis, prone to malignant transformation, transformation or lymphoma.
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