Have you ever thought of your body as a castle - strong, protective, but sometimes under siege when illness strikes? Castleman's disease may sound like something out of a fantasy tale, but for those living with it, the battle is very real. With its mysterious symptoms and unpredictable course, it can feel like fighting an invisible enemy within your own walls.^1,2

Yet, just like in every good story, there are strategies, allies, and tools to defend the castle. Managing Castleman disease isn’t about magic - it’s about knowledge, preparation, and the strength to keep moving forward.¹ With the right approach, long-term management becomes the key to unlocking stability and reclaiming control.

Castleman disease

Castleman disease (CD) is named after Benjamin Castleman, who first described the condition in 1957 as a localised enlargement of lymph nodes, initially recognised as a single-node disorder.³ It was later identified as a distinct disease entity, as a multicentric form was found, where multiple lymph nodes are affected, sometimes systemically. Giant lymph node hyperplasia and angiofollicular lymph node hyperplasia are other names for this disorder.³

CD is a rare disorder involving abnormal growth of lymph node tissue, which can lead to a range of immune and inflammatory responses in the body.⁴ It is not a type of cancer, but it shares some features with lymphomas and other immune-related conditions. It exists in two main forms:⁴ 

• Unicentric Castleman Disease (UCD) - Affects a single lymph node or single group of lymph nodes, often with no systemic symptoms. It tends to be localised, usually treated with surgery, and has a good prognosis 
• Multicentric Castleman Disease (MCD) - Involves multiple lymph node regions and can affect organs, causing systemic inflammation and immune dysfunction. MCD is often chronic, more serious, and requires ongoing treatment

The exact cause of CD is not fully understood.⁵ Some MCD cases are linked to infections, such as Human Herpesvirus 8, especially in people with weakened immune systems (e.g., HIV-positive individuals).⁵ Other cases are idiopathic, meaning the cause is unknown.⁵ Risk factors include immune suppression and certain viral infections. Common complications can include anemia, kidney problems, oedema, and an increased risk of infections.⁵

Symptoms vary depending on the type, but common features include:^1,5

• Enlarged lymph nodes
• Fatigue and weakness
• Fever and night sweats
• Unintended weight loss
• Swelling due to fluid buildup
• Enlarged liver or spleen (mainly in MCD)

Diagnosis typically involves blood tests, imaging (CT scans), and a lymph node biopsy, along with ruling out other conditions like infections or cancers.¹ Beyond the physical symptoms, living with CD can be challenging emotionally.⁶ Many patients face anxiety, depression, and uncertainty because of the disease’s unpredictable course and the need for ongoing management. These challenges can affect daily life, work, and relationships, making mental health support a vital part of comprehensive care.

Long-term medical management

Ongoing monitoring is essential in managing CD to track disease progression and adjust treatments as needed.¹ Regular follow-up visits allow healthcare providers to detect complications early and help maintain overall health. This follow-up care typically includes blood tests to monitor immune function and inflammation, imaging studies to evaluate lymph node size and organ involvement, and occasional biopsies if symptoms change or worsen.¹

Treatment strategies for CD vary depending on the type and severity.¹ Commonly, corticosteroids are used to reduce inflammation, immunosuppressive drugs help control the immune response, and chemotherapy may be necessary in more severe cases, especially in MCD.¹ Newer targeted therapies, like IL-6 inhibitors (for example, siltuximab and tocilizumab), specifically address the systemic inflammation seen in MCD.¹ Long-term medication usage can cause side effects such as increased risk of infections, fatigue or organ stress, managing these effects is a critical component of ongoing care.

UCD is frequently cured by surgery, which removes the afflicted lymph node. ¹Recovery involves monitoring for complications, managing pain, and follow-ups to ensure the disease does not recur or progress.

Lifestyle management

Maintaining a balanced diet is essential for supporting immune function and overall health in people with CD. Proper nutrition helps manage symptoms, supports recovery, and combats common issues like unintended weight loss and nutritional deficiencies.⁷ Working with a dietitian can help tailor dietary choices to individual needs.

Physical activity should be adapted to each person’s abilities and energy levels. Frequent exercise can increase mood, strengthen muscles, and lessen fatigue⁸ Even light activities like walking or stretching can make a positive difference in managing the disease’s impact on daily life.

It can be emotionally draining to live with a chronic illness such as CD.⁶ It is critical to create coping mechanisms in order to manage anxiety, depression, and uncertainty. Psychological support through therapy or counseling, as well as connecting with support groups, can provide comfort and guidance. Practices such as mindfulness, relaxation techniques, and engaging in hobbies also play a vital role in reducing stress and improving overall well-being.^9,10

Social & emotional well-being

The long-term management of CD heavily relies on family, friends, and caretakers. Their support can provide emotional comfort, practical help, and encouragement through treatment and daily challenges.¹¹ Connecting with patient support groups, both online and in-person, can also offer valuable community, shared experiences, and resources tailored to those living with the disease.

Managing social interactions while coping with CD can be difficult, especially when dealing with fatigue or unpredictable symptoms. It’s important to communicate openly with loved ones about needs and limits. Balancing responsibilities such as work, school, or family life with health care demands requires planning and sometimes flexibility, but maintaining social connections remains important for emotional well-being.¹¹

Managing the costs of CD, including treatments and ongoing care, can be challenging. In the UK, the National Health Service (NHS) provides most treatments free at the point of use, but some related expenses may still arise.¹² It’s important to understand your entitlement to benefits such as Personal Independence Payment (PIP) or Employment and Support Allowance (ESA) if your condition affects your ability to work. Employees with chronic illnesses have legal safeguards under the Equality Act 2010, which ensures that companies provide reasonable accommodations to support them at work.¹³ This helps safeguard your rights and access to necessary adjustments, promoting job security and a balanced work-life experience.

Emerging treatments & research

Ongoing clinical trials are exploring new treatments for CD, particularly for the more complex multicentric form.^14,15 These include advanced immunotherapies, novel biologic drugs targeting inflammatory pathways like IL-6, and therapies designed to modulate the immune system more precisely and with fewer side effects.

The involvement of immunological dysfunction and genetics in CD is also being studied.¹⁶ Promising areas include immunotherapy, which enhances the body’s natural defenses, and gene-based research, which may one day help identify the root causes of the disease or offer personalised treatment approaches.

While CD remains rare and complex, advances in medical research are offering new hope. As understanding of the disease deepens, so does the potential for more effective long-term treatments and possibly a cure. Continued research, patient advocacy, and clinical trial participation are all vital to driving progress forward.

FAQ’s

How long can you live with castleman's disease?

The life expectancy for someone with Castleman’s disease depends on the type and severity of the condition.¹⁷ Most people with UCD go on to live a normal lifespan with minimal risk of recurrence. MCD requires ongoing treatment. However, patients with MCD can live for many years, particularly with early diagnosis and consistent medical care. 

Can castleman's disease turn into lymphoma?

Castleman’s disease is not itself a form of cancer, but in some cases, particularly with MCD, there is an increased risk of developing lymphoma, a type of blood cancer.¹⁸ Certain rare lymphomas, such as non-Hodgkin lymphoma or plasmablastic lymphoma, have been observed in some patients with MCD. On the other hand, UCD carries a much lower risk of progressing to lymphoma.

Due to the potential risk, people with MCD are typically monitored closely with regular blood tests, imaging, and sometimes biopsies if symptoms worsen or change. Early detection of any malignant transformation is important for timely intervention. While lymphoma development is relatively uncommon, being aware of the possibility allows patients and healthcare providers to manage the disease more proactively.

Summary

Effective long-term management of CD requires a comprehensive, multidisciplinary approach. This includes regular medical monitoring, appropriate treatment, lifestyle adjustments, mental health support, and social care. Whether dealing with UCD or MCD, early detection, consistent follow-up, and proactive symptom management are essential for maintaining stability and quality of life. A holistic care plan that considers both physical and emotional well-being can help patients navigate the daily challenges of the disease. With access to the right information, support networks, and healthcare resources, individuals can live with greater confidence and resilience.

Patients and caregivers are encouraged to maintain ongoing communication with healthcare providers, report any changes in symptoms promptly, and discuss emerging treatment options or participation in clinical trials. Staying informed about the latest research can empower patients to make well-informed decisions about their care. Engaging with patient advocacy groups and support communities can offer valuable emotional support, practical advice, and a sense of connection with others facing similar experiences.