OVERVIEW
Overview of Castleman’s disease
Castleman’s disease (CD) is a rare disease of the lymph nodes and lymphoid tissues, which is pathologically characterized by marked hyperplasia of lymphoid follicles, blood vessels, and plasma cells to varying degrees, and clinically by marked enlargement of deep or superficial lymph nodes. Some cases may be accompanied by systemic symptoms and/or multi-system damage, and are reactive hyperplastic lesions of the lymphatic system caused by unexplained antigenic stimulation or immunoregulatory disorders.
Whether medical insurance
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Department
Hematology, Rheumatology
Synonyms
Giant lymph node hyperplasia, Castleman’s tumor, angiofollicular lymphoid hyperplasia, GLNH
Clinical Symptoms
Clinical manifestations are mostly asymptomatic, and may include fever, malaise, emaciation, liver and spleen enlargement. Lymphoid hyperplasia can occur anywhere lymph nodes are present.
Hazards
The multicentric form of the disease has the potential to lead to a life-threatening lymphoma.
Complications
Nephrotic syndrome, amyloidosis, myasthenia gravis, peripheral neuropathy, temporal arteritis, desiccation syndrome, thrombotic thrombocytopenic purpura, Kaposi’s sarcoma, B-cell lymphoma, etc.
Examination
Blood routine, bone marrow image, lymph node biopsy, CT, ultrasound, etc.
Diagnosis
Diagnosis can be made on the basis of obvious enlargement of lymph nodes, with or without systemic symptoms, and typical lymph node pathologic manifestations. Differences in clinical manifestations and pathologic manifestations are helpful for diagnosis.
Principles of treatment
Surgical excision of lesions in focal Castleman’s disease; chemotherapy or surgical excision of lesions in multicentric Castleman’s disease, depending on the condition of the lesions.
Curability
Surgery for focal Castleman’s disease has a good outcome; the prognosis of systemic multicentric Castleman’s disease (mainly the plasma cell type) is worse than that of single-unit Castleman’s disease, and many patients develop severe infections or even malignant lymphoma, which requires close follow-up.
Dietary advice
Eat a high-calorie, high-nutrition, low-protein diet and drink plenty of water.
Etiology
Epidemiology
Focal type is more common in young people.
Etiology
The etiology is not known. The plasma cell type is thought to be related to infection and inflammation, and HHV-8 infection is clinically confirmed in 25% of focal cases. It is also thought that at least some Castleman’s disease is at risk for B-cell malignant hyperplasia, and a minority of the multicentric type may convert to malignant lymphoma, but most cases do not convert to malignancy on follow-up.
Symptoms and diagnosis
Typical symptoms
The disease is clinically divided into focal and multicentric types.1. 90% of the pathologic manifestations of the focal type are of the hyaline vascular type. Clinical manifestation is painless enlargement of single lymph node, slow growth, forming a huge mass, which can occur in any part of lymphatic tissues, with mediastinal lymph nodes being the most common, followed by cervical, axillary and abdominal lymph nodes, and occasionally in extra-nodal tissues. Most of them have no systemic symptoms and have a benign course. 10% of the cases are of plasma cell type, and involvement of abdominal lymph nodes is common, which is often accompanied by systemic symptoms, such as prolonged hypothermia or hyperthermia, malaise, lethargy, anemia, etc. 2. Multicentric type is less common. Patients have multi-site lymph node enlargement, easily spread to superficial lymph nodes, accompanied by systemic symptoms, such as fever and hepatosplenomegaly, often with multi-systemic involvement, such as nephrotic syndrome, amyloidosis, myasthenia gravis, peripheral neuropathy, temporal arteritis, Sjögren’s syndrome, thrombotic thrombocytopenic purpura, as well as oral cavity, corneal inflammatory reaction. In a few patients, Kaposi’s sarcoma or B-cell lymphoma may complicate the disease. In addition, the multicentric form often has an aggressive clinical course and is prone to infections.
Other symptoms
In a few patients, multiple neuropathies, organomegaly (liver, spleen), endocrinopathy, skin lesions, and serum immunoglobulin monoclonalities may be present at the same time.
Diagnostic basis
1. Preliminary diagnosis can be made on the basis of obvious enlargement of lymph nodes with or without systemic symptoms, which often include fever, malaise, weight loss, anemia, elevated blood sedimentation, increased blood gammaglobulin and hypoalbuminemia, etc. 2. Confirmation of the diagnosis requires typical Castleman’s disease pathological changes in lymph node biopsy. Pathologic changes can provide a basis for the diagnosis of subtypes. (1) Transparent vascularized type can be seen in the lymph nodes with many enlarged lymphoid follicle-like structures, which are scattered. There are several small blood vessels penetrating into the follicles, the endothelium of the blood vessels is obviously swollen, the wall is thickened, and the later stage is glassy. (2) In the plasma cell type, follicular hyperplasia can be seen in the lymph node microscopically, with small blood vessels penetrating into the follicles, and the lymphocyte hyperplasia around the follicles is much less obvious than that of the hyaline vascular type, and there is generally no typical onion skin-like structure.
Treatment
Treatment guidelines
Surgical resection for focal lesions; surgical resection for multicentric lesions that invade only a few areas, followed by chemotherapy or radiotherapy.
Radiotherapy
Chemotherapy or radiotherapy can be added after surgery for multicentric type; chemotherapy is the only choice for multicentric type with extensive lesions, or local radiotherapy can be added to the main lesions, but most of them can only get partial remission. Chemotherapy is usually chosen as the combination chemotherapy for malignant lymphoma.
Surgical treatment
1. Surgical resection of focal lesions. If the pathology is of plasma cell type, if accompanied by systemic symptoms, the symptoms can be rapidly disappeared after removing the diseased lymph nodes. 2. Multi-center type lesions can also be surgically removed if the lesions involve a few areas.
Other treatments
Autologous hematopoietic stem cell transplantation may be an option for the treatment of multicentric Castleman disease.
Prognosis
Focal Castleman disease has a good prognosis; multicentric Castleman disease with monoclonal hypergammaglobulinemia has a poorer prognosis and is prone to malignancy.
Nursing care
Daily care
1. During chemotherapy, pay attention to the protection of venous blood vessels and observe the body’s reaction after drug administration. 2. Observe the local reaction of skin after radiotherapy, avoid strong hot or cold stimulation, and avoid the use of irritating objects. 3. Wear loose, soft cotton clothes.3. Bed rest for those with fever, and keep the temperature and humidity of the room suitable.4. Pay attention to the oral cavity, perineum, perianal cleanliness, and prevent infections.5. Maintain a comfortable mood, balanced nutrition, exercise, and improve the immunity of the body.6. Follow the instructions of the doctor for regular follow-up.
Dietary management
Eat a high-calorie, high-nutrition, low-protein diet and drink plenty of water.