Castleman’s disease is a rare disorder of the lymph nodes and lymphatic tissues. It is also called lymphoproliferative disorder, giant lymph node hyperplasia, and angiofollicular lymph node hyperplasia.
Usually benign, it is not a cancer per se, but one of its subtypes, the multicentric form, behaves like a lymphoma. Additionally, many Castleman’s disease patients eventually develop lymphomas later on.
The causes and risk factors of Castleman’s syndrome are not well known, and there is also no reliable data on its incidence in Brazil or worldwide. It is known, however, that there is a higher likelihood of occurrence in HIV-infected patients, and that the disease can affect both children and adults.
An important aspect of Castleman’s disease to highlight is that it can severely weaken the immune system, making it more difficult to fight infections.
Types of Castleman’s Disease
Two forms of the disease are known:
- Localized Disease – affects only one group of lymph nodes – most often from the chest or abdomen – and is not widespread. The affected lymph nodes enlarge and may press on other organs or tissues in the area where they are located; and
- Multicentric Disease – affects multiple groups of lymph nodes and can involve other organs containing lymphoid tissue. It is more severe than the localized form, especially in patients with HIV.
There are also microscopic subtypes of Castleman’s disease – when it is classified according to the form of lymphoid tissue:
- Hyaline-vascular type – the most common, usually localized;
- Plasma cell type – more likely to be multicentric;
- Mixed type – the least common, with areas of both localized and multicentric types.
- Finally, Castleman’s disease can be classified based on viral infections:
- HIV;
- Human herpesvirus-8 (HHV-8).
Symptoms of Castleman’s disease
Localized Castleman’s disease tends to present symptoms such as:
Respiratory problems caused by compression on the trachea or bronchi, when the enlargement occurs in the lymph nodes of the chest; and
Abdominal pain and difficulty swallowing, caused by the enlargement of abdominal lymph nodes.
In the multicentric subtype, the disease can present with the presence of nodules under the skin, especially in areas like the neck, groin, or armpits, in addition to those mentioned above.
Some symptoms are common to both forms of Castleman’s disease:
- Infections;
- Fever;
- Unexplained weight loss;
- Fatigue;
- Night sweats; and
- Weakness and/or numbness (caused by nerve damage).
Diagnosis
Diagnosis of Castleman’s disease is made through biopsy. Patients often seek medical attention due to various health problems such as gastrointestinal or respiratory disorders. Physical examination or imaging typically reveals enlarged lymph nodes that can be biopsied to confirm the diagnosis.
Such changes are not definitive for the diagnosis, as they are common in infections and anemia, for example. Therefore, the doctor will request imaging tests, such as:
- Computed tomography – to check in detail the soft tissues and whether the lymph nodes or internal organs are enlarged;
- Magnetic resonance imaging – especially if the doctor is concerned about areas near the spinal cord or brain;
- Chest X-ray – to identify if there are lymph nodes in the chest affected by the disease (usually requested when the patient presents respiratory problems as a symptom);
- Abdominal ultrasound – to identify if there are lymph nodes in the abdomen affected by the disease; and
- PET-Scan – useful for finding small growths in the lymph nodes and lymphatic tissues that do not appear on computed tomography.
Treatment
The treatment approaches for Castleman disease include surgery, radiation therapy, and chemotherapy, among other medications.
In the case of localized Castleman disease, patients are usually cured when the affected lymph nodes are removed through surgery. If the issue cannot be fully resolved with surgical intervention, either due to the patient’s overall health conditions or the inability to completely remove the affected lymph nodes, radiation therapy is considered. The disease tends not to return.
On the other hand, cases of multicentric Castleman disease HHV-8 negative and HIV negative can be treated with anti-IL-6 monoclonal antibodies. Some patients may require treatment with chemotherapy in association with anti-CD20 monoclonal antibodies. In case of association with POEMS syndrome, treatment follows the guidelines for treating this syndrome. If the patient has HIV, it is necessary to combine the treatment with the medications already used to control the infection.
Prevention of Castleman Disease
Since the cause of Castleman disease is unknown, there are no known ways to prevent it.
