Introduction

If you want to know more about unicentric Castleman disease, you’re in the right place. We have the answers you’re looking for to make sure you are equipped to recognise the early signs for effective management. This article will provide a clear and concise overview of the common symptoms of this disease, offering clarity and understanding.

Castleman's disease is a group of rare and heterogeneous disorders with lymph node abnormalities.¹ It typically involves a hyperactive immune system, such as lymph nodes, which remain activated even after the threat is gone, leading to inflammation and damage to organs. This can lead to swollen lymph nodes as well as there being changes to its tissue. There are two types of Castleman disease, thus symptoms can be presented differently. Unicentric Castleman disease typically involves the enlargement of a single lymph node or a localised group of lymph nodes, whereas multicentric Castleman disease is characterised by lymph node enlargement in multiple regions throughout the body. However, the focus of this article will be on unicentric Castleman disease to ensure the symptoms are clearly understood as well as to prevent the disease progressing.

What are the general characteristics of unicentric castleman disease?

Most people with this disease don’t notice any unusual signs or symptoms, with the enlarged node in only one region of the body being recognised during a physical exam for a different problem.² For some patients, the enlarged lymph node can create pressure onto a nearby organ such as the chest, neck, or abdomen, causing mild symptoms usually seen in multicentric Castleman disease. Thus, diagnosis of this disease cannot be made with symptoms alone, with further testing required.

Less commonly, some patients can experience inflammatory symptoms such as fatigue, with laboratory examinations showing low levels of haemoglobin.

What are localised symptoms?

An enlarged lymph node can cause many different effects, such as palpable, painless swelling in the neck, armpit or groin. Lesions in the chest may present with a cough and cause chest discomfort. Whereas lesions in the abdomen and neck can cause back or abdominal discomfort, pain, bloating as well as changes in voice and difficulty in swallowing.²

What are systemic symptoms seen in patients?

Symptoms such as those seen in the flu can be similar to unicentric which is why diagnosis can be quite difficult. Signs and symptoms seen in multicentric Castleman disease can be present in individuals with unicentric Castleman disease. This can include:

Body symptoms

• Fever
• Unintended weight loss
• Enlarged spleen or liver
• Night sweats
• Nausea and vomiting
• Swelling in the feet, or abdomens
• Numbness in hands and feet

Blood symptoms

• Anaemia
• High or low platelet counts
• Higher creatine due to kidneys not functioning as well
• Higher levels of immunoglobulins, a type of antibodies
• Low levels of albumin

What are laboratory findings to confirm this disease?

Castleman's disease can be difficult to diagnose as it is rare and presents itself similarly to other diseases. Physicians often need to rule out many diseases before testing is carried out to confirm it. Although there are various types of this disease, they all share changes to the lymph node that can be observed through a microscope as well as through other tests.

Further testing is needed to accurately diagnose this disease and determine the type. This may include:

• Lab tests: this will check for abnormal blood cell counts of haemoglobin, white blood cells, platelets as well as inflammation markers and markers of organ dysfunction such as liver function tests, albumin and creatinine
• Imaging tests: CT and PET scans can locate enlarged lymph nodes or other organs that are also affected, such as liver or spleen
• Lymph node biopsy: This is the only way to confirm this disease. The lymph node is surgically removed and a sample of it is examined under a microscope so that it can be evaluated by a pathologist. From this, the enlarged lymph node can be detected as well as in its specific region

When should I suspect that I have unicentric castleman disease?

If a lump is found in the neck, armpit or groin, it is important to contact a healthcare provider as well as if other symptoms of Castleman disease are present and persist for a few weeks. For example, symptoms such as a lasting feeling of fullness in your chest or abdomen as well as fatigue, fever or unexplained weight loss, should be reported by a healthcare professional immediately.

Summary

Castleman's disease is a rare disorder, usually caused by enlarged lymph nodes. Different symptoms are often presented but this can vary depending on the type. For unicentric Castleman disease, the symptoms are often unexplained weight loss, fatigue, nausea and vomiting, with other effects occurring due to pressure on nearby organs. The disease can be diagnosed through different testing such as lab tests checking for blood cell counts and CT scans locating the lymph nodes. However, a lymph node biopsy is most effective in examining the lymph node and confirming the disease. Thus, it is essential to understand the common signs and symptoms of unicentric Castleman disease to ensure effective treatment is given to the patient before it progresses.

FAQs

What are the treatments for this disease?

Treatments of this depends on the type and can include radiotherapy, steroids, tocilizumab, rituximab, immunosuppressive agents, as well as chemotherapy.³ However, the optimal treatment for unicentric Castleman disease is surgery, with there being clear evidence of this being the most effective.⁴ This usually involves a complete surgical removal of the lymph nodes which not only confirms the diagnosis but can also cure the condition if the entire lymph node is removed. If multiple, nearby lymph nodes are involved, known as a cluster, a more extensive procedure known as locoregional systematic lymphadenectomy is done, which removes all the lymph nodes in that region to ensure the disease is fully cleared.²

Radiation or immunotherapy is sometimes used before surgery to shrink the lymph node, making it easier for doctors to remove it completely.

At what age are patients usually diagnosed?

Patients can be diagnosed at any age, ranging from 2 to 84 years old with the median age of diagnosis being 34 years, with a greater proportion being in females.¹

What is the prognosis for people with UCD?

It is typically good for most people, with removal of the lymph node often enough being able to cure it.

Can unicentric castleman disease become multicentric?

Sometimes this can occur such as infection with HHV-8 is a risk factor for MCD, particularly in patients with a weak immune system as this can increase the number of enlarged lymph nodes.⁵