Giant lymph node hyperplasia, known as Castleman disease, marks by big lymph nodes. It shows as either unicentric Castleman disease or multicentric Castleman disease.
UCD is often in one place and minimally affects you, showing as swollen lymph nodes. In contrast, MCD is throughout the body, causing fever, night sweats, and weight loss.
Its exact cause remains a mystery. But, experts believe it comes from ongoing mild inflammation. A key part in this is the cytokine IL-6, linked to immune reactions and inflammation.
To diagnose giant lymph node hyperplasia, a lymph node biopsy is needed. This helps find the disease’s telltale signs. Doctors may also order blood tests, look for inflammation, and do scans to confirm.
For MCD, treatments include surgery, chemo, and drugs like prednisolone. There are also antiviral methods and drugs that target CD20 or IL-6. When these don’t work, stem cell therapy might help in MCD or POEMS syndrome cases.
Key Takeaways:
- Giant lymph node hyperplasia, or Castleman disease, is a rare lymphoproliferative condition characterized by enlarged lymph nodes.
- There are two main forms of the disease: unicentric Castleman disease (UCD) and multicentric Castleman disease (MCD).
- UCD is localized and often presents with minimal symptoms, while MCD is a systemic disease with more significant symptoms.
- Chronic low-grade inflammation and interleukin-6 (IL-6) are believed to play a role in the development of the disease.
- Treatment options for MCD include surgery, chemotherapy, prednisolone, antiviral strategies, monoclonal antibody therapies, and stem cell therapy for non-responsive cases.
Types and Symptoms of Giant Lymph Node Hyperplasia
Giant lymph node hyperplasia comes in two main types. These are unicentric Castleman disease (UCD) and multicentric Castleman disease (MCD). Knowing the differences is key for the right diagnosis and treatment.
Unicentric Castleman Disease (UCD)
UCD is a type that affects just one lymph node or a few nearby ones. It’s common in younger people. Often, there are no signs or only a few mild ones.
The main sign of UCD is painless swelling of lymph nodes in the neck, chest, or stomach. Sometimes, you can feel these swollen nodes as lumps under the skin. If they press on nearby areas, you might feel pain or discomfort.
Sometimes, UCD can turn into a more serious form. This is called unicentric Castleman disease with vascular proliferation (UCD-VP). It involves a lot of blood vessels growing in the enlarged lymph nodes.
Multicentric Castleman Disease (MCD)
MCD is a more serious type. It enlarges many lymph nodes all over the body. This type is often found in older people.
The symptoms of MCD can differ from person to person. But, the most common ones include:
- Recurrent or persistent fevers
- Night sweats
- Fatigue
- Unintentional weight loss
MCD can also cause lymph node enlargement, liver and spleen enlargement, and fluid retention. This last one leads to swollen legs or ankles.
It’s important to know that MCD symptoms can change based on the subtype and the presence of other diseases like POEMS syndrome.
Diagnosis and Treatment of Giant Lymph Node Hyperplasia
To diagnose giant lymph node hyperplasia, a series of tests are used. A biopsy is done to find its specific features. This is key in telling it apart from other conditions.
Tests like X-rays and PET-CT scans show the size and place of swollen lymph nodes. They also reveal how far the disease has spread.
For multicentric Castleman disease, checking for HHV-8 or HIV is important. Finding these infections helps to diagnose and treat the disease.
The cure for giant lymph node hyperplasia varies based on its type and how bad it is. In unicentric Castleman disease (UCD), surgery to take out the affected lymph node(s) helps a lot.
If it’s multicentric Castleman disease (MCD), treatment may include surgery, chemo, and prednisolone. These aim to calm the immune reaction causing MCD. Antivirals fight HHV-8 or HIV, if need be.
Drugs like monoclonal antibodies, against CD20 or IL-6, can also be used for MCD. They stop certain proteins, helping to control the disease. This can make a big difference for the patient.
If the usual treatments don’t work, stem cell therapy might be an option. New, healthy cells are put in to replace the ones that are not working well. This treatment is a new hope for those hard to treat cases.
Treatment Options for Giant Lymph Node Hyperplasia
| Treatment | Description |
|---|---|
| Surgery | Removal of affected lymph node(s) in unicentric Castleman disease |
| Chemotherapy | Systemic treatment to suppress abnormal immune response in multicentric Castleman disease |
| Prednisolone | Corticosteroid used in combination with chemotherapy to manage symptoms and reduce disease activity |
| Antiviral strategies | Targeting HHV-8 or HIV infections associated with multicentric Castleman disease |
| Monoclonal antibody therapies | Targeting CD20 or IL-6 to neutralize proteins involved in disease pathogenesis |
| Stem cell therapy | Transplantation of healthy stem cells as a potential treatment option |
Conclusion
Castleman disease is a rare condition that causes swollen lymph nodes. It appears in two forms: unicentric Castleman disease and multicentric Castleman disease.
UCD is limited and often doesn’t show strong symptoms. MCD, however, spreads throughout the body, affecting many lymph nodes. It can cause various symptoms and issues. The main cause of this disease is not certain. It likely involves ongoing low-grade inflammation and the IL-6 substance.
Treatments include surgery, chemo, prednisolone, antiviral methods, and drugs targeting specific proteins. If these don’t work, using stem cells might be an option. Scientists are working hard to find better treatments that will help patients with this condition live better lives.