The term "Castleman’s disease" refers to the pathological process of lymphoproliferative nature. The clinical picture of the disease and its outcome directly depend on the localization of the affected lymph nodes and its histological type. Castleman's disease (a photo of a part of the body over the affected area is presented below) is an extremely rare pathology. It is diagnosed in 2 people out of a hundred thousand. For the first time, the disease became known in 1956. Malignancy is not typical for the initial stage of development of pathology. However, over time, the disease can degenerate into an oncological disease. Currently, there are both conservative and surgical methods of treating the disease.
Pathogenesis and etiology
To date, scientists do not have information on the development mechanism and causes of Castleman’s disease. After conducting numerous studies, we can only conclude that, perhaps, a direct involvement in the occurrence of pathology is taken by the herpes virus type 7 and 8. It is a pathogen that is also associated with the development of Kaposi’s sarcoma.
In the International Classification of Diseases (ICD-10), Castleman’s disease is assigned code D47.7.
Hyaline-vascular type of pathology
This form of the disease is most often diagnosed in young people. It is characterized by damage to the mediastinal lymph nodes. In some cases, degenerative changes affect peripheral areas.
Castleman's disease of a hyaline-vascular type is usually an isolated lesion. The course of the pathology in most cases is not accompanied by the occurrence of systemic manifestations. The disease is often combined with the formation of tumors.
Most often, the hyaline-vascular type of Castleman's disease is asymptomatic. As a rule, pathology is detected randomly during a routine medical examination.
The manifestations of Castleman's disease of this type are nonspecific. The main symptoms of the disease:
- Pain in the chest or in the epigastric zone.
- Increased fatigue.
- Weight loss.
- Mild fever.
- An increase in the size of peripheral lymph nodes. The skin above them does not change, they are dense, painless and inactive.
For this form, only a localized version of the flow is characteristic.
Plasma cell type of disease
It is diagnosed in people of all ages. Most often, this type of Castleman disease manifests itself in the form of lymphadenopathy with damage to both one and a whole group of lymph nodes. In this case, the clinical manifestations are systemic.
Symptoms of plasma-type Castleman disease:
- Polyneuropathy (uncontrolled trembling of the extremities, extremely painful muscle crumps, fasciculations, a feeling of "goosebumps" on the skin).
- Organomegaly (this term refers to an abnormal increase in internal organs in size).
- Endocrinopathy (a sharp decrease in body weight, deterioration of the skin, lethargy, apathy, impaired appetite, nausea, arterial hypotension, visual impairment, migraine, mental disorders).
- Monoclonal gammopathy (bone loss).
In addition, laboratory indicators also change significantly. During a blood test, anemia and an increased ESR are detected.
Mixed type
It is customary to talk about it if it is not possible to clearly determine the form of pathology. In other words, patients exhibit symptoms of both the hyaline-vascular and plasma cell types.
Unicentric version of the flow
In other words, this is a localized form of the disease. It accounts for 90% of all cases. In individuals under 30 years of age, the plasma cell type is predominantly diagnosed. In people from 30 to 40 years old, the hyaline-vascular type of Castleman disease is most often detected.
The main clinical manifestations of a localized form of pathology:
- The increase in lymph nodes in size in the abdominal cavity and mediastinum.
- Anemia.
- The increase in internal organs in size.
In some cases, the pathology is asymptomatic. The patient is only occasionally disturbed by discomfort in the chest or epigastric zone. Sometimes patients note that the internal tissues of their body seem to be squeezed by the affected lymph nodes.
A feature of this variant of the course is that it is not associated with HIV among doctors. It is extremely rare in patients with herpes virus to be detected in the blood.
Multicentric type
This variant of the course is diagnosed mainly in individuals whose age is from 50 to 60 years. The multicentric type is considered the most dangerous. In most cases, pathology is a threat not only to health, but also to the life of the patient.
The main clinical manifestations of the disease:
- An increase in the size of the liver and / or spleen.
- Reduced degree of performance.
- Fever.
- Constant feeling of tiredness.
- Anemia.
- Excessive perspiration, especially at night.
- A sharp decrease in body weight.
Fortunately, the multicentric type is found in only 10% of patients. It is characterized by the defeat of both central and peripheral lymph nodes.
A third of patients with the multicentric type of Castleman disease suffer from HIV. At the same time, the herpes virus is detected in all.
The course of the disease is characterized by frequent relapses. A persistent period of remission is extremely difficult to achieve. In addition, over time, the disease can transform into a malignant lymphoma. The prognosis in most cases is unfavorable.
Laboratory diagnostics
If there are alarming symptoms (the most specific of them is an increase in lymph nodes), you need to contact an infectious disease specialist. If necessary, he will send a consultation with an oncologist, TB specialist or surgeon.
Primary diagnosis involves a medical history and physical examination of the patient. During palpation, the doctor reveals enlarged lymph nodes, assesses their localization and consistency. In all cases, pronounced splenomegaly is detected.
Based on the results of the initial diagnosis, the doctor sends the patient to the laboratory for delivery of biomaterial. Necessary research:
- Clinical blood test. As a rule, leukocytosis, thrombocytosis, anemia, and an increase in ESR are detected in it.
- Analysis of liquid connective tissue by polymerase chain reaction. The purpose of the study is to confirm or exclude the presence of herpes viruses type 7 and 8 in the blood. Currently, scientists believe that they are true triggers for the development of Castleman disease.
- Blood chemistry. The following indicators are clinically significant: albumin, total protein, bilirubin, urea, creatinine, ASAT, ALAT, LDH, GGT, glucose, alkaline phosphatase, C-reactive protein.
- A blood test for immunoglobulins of classes A, M and G. Most often, their increase is observed against the background of a multicentric variant of the pathology.
- Blood test for hormones. The most significant are the following indicators: cortisol, TSH, T3 and T4.
- Determination of blood type and Rh factor.
- Analysis of fluid connective tissue for HIV.
- General urine test. Clinically significant indicators: specific gravity, glucose, protein. In addition, a urine sediment is examined under a microscope.
Based on the results of laboratory tests, the doctor can make a preliminary diagnosis. To clarify it, it is necessary to evaluate the results of a comprehensive survey.
Instrumental diagnostics
As a rule, doctors prescribe:
- Thermometry
- Regular measurement of peripheral blood pressure.
- Bronchoscopy.
- Diagnostic laparoscopy.
- Thoracoscopy
- Esophagoduodenoscopy.
- Ultrasound of the abdominal cavity, lymph nodes, kidneys, soft tissues of the thyroid gland.
- MRI of bone structures.
- ECG.
- ECHOKG.
- X-ray examination.
- CT
- Lymph node biopsy.
- Positron emission tomography.
Based on the results of a comprehensive diagnosis, the doctor draws up the most effective treatment regimen for Castleman disease.
Conservative therapy
Currently, it is effective only for patients suffering from the multicentric course of pathology. The treatment regimen consists of the following items:
- Intravenous administration of Cyclophosphamide. This is an antitumor drug that also has an immunomodulating effect.
- Introduction of Vincristine. It is also an antitumor agent.
- Introduction "Doxorubicin". This is an antibiotic that has not only antiproliferative, but also antimitotic effects.
- Intravenous or drip administration of etoposide. This is an antitumor drug that has a cytotoxic effect.
- Introduction of Vinblastine. Antitumor agent of plant origin.
- Introduction of "Tocilizumab." The active component is an antibody to the human IL-6 receptor.
- Introduction of Bortezomib. It is an inhibitor of the activity of the 26S proteasome.
- Introduction "Ruxolitinib". It is an antitumor drug that is also a protein kinase inhibitor.
- Introduction of Ondasetron. This remedy is antiemetic. The active component of the drug prevents the appearance of nausea, which is a side effect of chemotherapy.
- Introduction of Prednisolone. This drug has a beneficial effect on the functioning of the organs of the endocrine system.
- Reception "Furosemide." This drug is a powerful diuretic.
If necessary, the doctor prescribes the introduction of electrolytes and solutions as concomitant therapy. In addition, he can recommend taking NSAIDs, antibiotics, antiviral and antifungal agents.
Surgical intervention
If Castleman's disease is localized, conservative treatment methods will not lead to a positive result. In this case, the operation is indicated. During the surgical procedure, a resection of the affected lymph node is performed. The excised tissues are then sent to the laboratory for histomorphological and immunohistological analysis.
In some cases, the doctor may prescribe radiation therapy and stem cell transplantation.
Forecast
Castleman's disease is an extremely serious and dangerous pathology. Its outcome directly depends on the form of the disease and the timeliness of contacting a doctor.
At the initial stage of development, the disease is benign. In the absence of competent treatment, it can degenerate into lymphoma. This is true for all patients with any form of ailment.
With multicentric pathology, the prognosis is the most unfavorable. According to statistics, a fatal outcome occurs within a few months from the onset of the disease. This is due to the fact that this form of the disease is most often diagnosed in people with HIV. In addition, it often leads to the development of multiple organ failure and cancer.
With the unicentric variant of the course of the disease, the prognosis is more favorable. As a rule, recovery occurs in most patients after removal of the affected lymph node. But at the same time, the risk of developing lymphoma remains.
Prevention
Currently, there are no measures whose implementation significantly reduces the likelihood of a dangerous disease. In addition, preventive regimens have not been developed for patients with previously diagnosed pathologies. Such persons only need to visit doctors regularly (once every six months for 3 years).
It is important for patients who have already been diagnosed with Castleman's disease in the past to consult with an oncologist, neurologist and ophthalmologist. In addition, they need to regularly donate blood and monitor their blood pressure.
Finally
Castleman's disease is a serious pathology of a lymphoproliferative nature. It is characterized by the defeat of one or a whole group of lymph nodes. The disease can be localized or multicentric. In the first case, the prognosis is more favorable, in most patients, after surgery, recovery occurs. Life expectancy with a multicentric form is only a few months.
In ICD-10, Castleman's disease is assigned code D47.7.