17920 Castleman Disease

Overview

Castleman disease is a rare group of conditions that affect the lymph nodes, the small bean-shaped glands that help your body fight infection. In Castleman disease, certain lymph nodes grow abnormally and can cause symptoms throughout your body. There are two main types: unicentric (affecting one group of lymph nodes) and multicentric (affecting many groups of lymph nodes).

No. Castleman disease is very rare. Most doctors will never see a case during their career, and exact numbers are hard to know.

It can occur at any age, but it is most often diagnosed in people between 35 and 50 years old. The unicentric type is more common in younger people, while the multicentric type tends to affect older adults and people with weakened immune systems.

Symptoms

Common symptoms

• Swollen lymph nodes, often in the neck, armpit, or groin
• Fever
• Night sweats
• Unexplained weight loss
• Feeling very tired or weak

Symptoms in children

• The same swollen lymph nodes, fever, and fatigue
• Poor appetite or slower growth than expected
• Being more fussy or irritable than usual

Symptoms in older adults

• More severe fatigue and weakness
• Fevers and night sweats that come and go
• Confusion or dizziness
• Unintentional weight loss that may be mistaken for ageing or another health problem

Causes

Main causes

• The exact cause of Castleman disease is unknown.
• It is not passed down from parent to child in most cases.
• Some cases are linked to a viral infection called HHV-8, a type of herpes virus.
• People with HIV or other conditions that weaken the immune system are more likely to get the multicentric type.

Risk factors

• Having a weakened immune system, such as from HIV/AIDS
• Being infected with HHV-8
• Being over 50 years old for the multicentric type
• Being male (slightly higher risk)

When to see a doctor

See a doctor urgently if:

• See a doctor the same day if you have a fever of 100.4°F (38°C) or higher with shaking chills
• See a doctor the same day if a lymph node becomes painful, red, or hot to the touch
• See a doctor the same day if you have significant unexplained weight loss or extreme fatigue

Book a routine appointment if:

• Make an appointment if you have a swollen lymph node that does not go away after a few weeks
• Make an appointment if you have ongoing fever, night sweats, or fatigue without any clear reason
• Make an appointment if you notice a lump that feels hard or fixed in place

Diagnosis

A doctor confirms Castleman disease with a lymph node biopsy. This means taking a small piece (or sometimes the whole) lymph node and looking at it under a microscope. Imaging scans and blood tests help check whether other lymph nodes are involved.

Tests that may be done

• Lymph node biopsy
• Blood tests to check for anaemia, inflammation, and organ function
• CT, MRI, or PET scans to look for other affected lymph nodes
• Tests for HIV and HHV-8 if needed
• Bone marrow biopsy in some cases

What to expect at your appointment

A biopsy is usually done as a short procedure with local or general anaesthetic. You may feel sore for a few days, but you can usually go home the same day. Results can take a week or two. If the diagnosis is multicentric Castleman disease, your doctor will refer you to a specialist in blood disorders or cancer care.

Treatment

Treatment depends on the type and severity of Castleman disease. For unicentric disease, surgery to remove the affected lymph node is often the main treatment and can be curative. For multicentric disease, treatment focuses on controlling symptoms and reducing the activity of the disease. Your specialist team will create a plan tailored to you.

Self-care at home

• Rest as needed and pace your daily activities
• Keep a symptom diary to share with your care team
• Talk to your doctor before receiving any vaccines
• Avoid close contact with people who have active infections
• Wash your hands regularly
• Eat a balanced diet to help your body cope with treatment

Medical treatments

Medical treatment for multicentric Castleman disease may include medicines that calm the immune system (immunosuppressants), steroids to reduce inflammation, and therapies that target specific proteins involved in the disease. In some cases, antiviral medicines are used if a viral trigger is found. More aggressive cases may be treated with chemotherapy or a stem cell transplant.

When is surgery considered?

Surgery is the main treatment for unicentric Castleman disease. If the entire affected lymph node is removed completely, the condition is often cured. If surgery is not possible, doctors may use radiotherapy or monitor the lymph node if it is very small.

Living with this condition

Living with Castleman disease can be challenging, especially when you have the multicentric type. Many people have good and bad periods. Keep a list of your medications and appointments, report new symptoms early, and ask for help when you need it.

Lifestyle tips

• Create a steady sleep routine to fight fatigue
• If you smoke, talk to your doctor about stopping
• Try to avoid large crowds during flu season
• Talk to your employer about flexible work if you need time off
• Stay active in a gentle way, like walking or stretching

Diet and exercise

Eat a balanced diet with plenty of fruits, vegetables, and protein to give your body strength. Gentle exercise like walking can improve energy, but listen to your body and rest when you need to. If you are losing weight or your appetite is poor, ask your doctor about seeing a dietitian.

Mental health and emotional wellbeing

A rare disease diagnosis can be emotionally difficult. It's normal to feel scared, anxious, or low. It may help to talk with a counsellor or join a support group. If you ever have thoughts of harming yourself, contact your local emergency number or a mental health crisis service immediately.

Prevention

Because the cause is not fully understood, Castleman disease cannot be prevented. However, for people living with HIV, keeping the virus under control with regular medical care may lower the risk of developing the multicentric form.

Vaccines

Stay up to date with routine vaccines, but always ask your doctor which vaccines are safe for you. Some live vaccines are not recommended for people with weakened immune systems.

Screening programmes

There is no routine screening test for Castleman disease. Regular medical check-ups are helpful, but screening is not recommended for the general public.

Complications

If left untreated

• Infections that may become severe or life-threatening
• Organ damage, including kidney or liver failure
• Severe inflammation that may affect the lungs, heart, or nervous system
• The multicentric type may progress to lymphoma or another blood cancer

Long-term outlook

The outlook depends on the type and your overall health. Unicentric Castleman disease is very often cured with surgery, and most people live normal lives. Multicentric disease is serious, but treatment has improved greatly. Many people achieve long periods of remission and continue to live active, fulfilling lives. Your doctor can give you a more personal outlook based on your situation.

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