Castleman syndrome in children causes symptoms and treatment

Castleman syndrome in children is a rare condition that causes significant concern for parents due to its impact on the lymph nodes and the immune system. Many moms and dads often ask: What causes this syndrome? How can I recognize its symptoms in my child? And is there an effective treatment that can help them live a normal life? In this article from Dalily Medical, we will explain all the details about the causes of Castleman syndrome in children, its symptoms, types, diagnostic methods, the latest treatment options, and essential tips for parents on how to manage the condition.

What is Castleman Syndrome?
Castleman Syndrome is a rare disease that affects the lymph nodes, causing them to enlarge abnormally. This can weaken the immune system and may also affect other organs in the body.
Is Castleman Syndrome a type of cancer?
No, Castleman Syndrome is not cancer. However, in some cases, its symptoms may resemble tumors, and it can increase the risk of certain cancers if left untreated.
Is the syndrome contagious?
No, Castleman Syndrome is not infectious. Although it is sometimes associated with human herpesvirus 8 (HHV-8), this does not mean it spreads from child to child.
What is the difference between unicentric and multicentric types?

Unicentric (UCD): Affects only one lymph node or one area.
Multicentric (MCD): Affects multiple lymph nodes and organs and is more severe.

What are the symptoms in children?

Swollen lymph nodes
Significant weight loss
Fatigue and low energy
Recurrent fevers
Night sweats
Loss of appetite

How is Castleman Syndrome diagnosed?

Blood tests
CT scans or MRI
Lymph node biopsy to confirm the diagnosis

Is there a definitive cure?

Unicentric type: Usually treated with surgery to remove the affected lymph node.
Multicentric type: Requires immune-modulating drugs, corticosteroids, or sometimes chemotherapy.
Continuous follow-up is essential to prevent complications.

Can a child live a normal life?
Yes, with proper treatment and regular monitoring, many children—especially with the unicentric type—can live a normal or near-normal life.
Can the syndrome recur after treatment?
Yes, especially in the multicentric type, which is why ongoing medical follow-up is crucial.
Do nutrition and vitamins help in treatment?
Healthy nutrition and vitamins support the immune system and help the child stay stronger, but they do not replace medical treatment.
At what age can the syndrome appear?
It can appear at any age, even in infants, though most cases occur in late childhood or early adolescence.
Does Castleman Syndrome affect growth?
Yes, some children may experience poor appetite, weight loss, and delayed growth if untreated.
Is there a genetic test for the syndrome?
Currently, there is no specific genetic test, but research is ongoing into genetic factors linked to the disease.
Are vaccinations safe for affected children?
Generally, standard vaccinations are safe, but the doctor should decide based on the child’s immune status, especially if taking immunosuppressive drugs.
Are there specific foods to eat or avoid?
There’s no special diet, but a healthy diet with proteins, vegetables, fruits, and vitamin-rich foods is recommended to support immunity.
Can the child attend school normally?
Yes, if the condition is stable and under treatment. Children with weakened immunity should take precautions against infections.
Are surgeries risky for affected children?
If the unicentric type is diagnosed early, surgery to remove the affected lymph node is usually very safe and successful.
Does the syndrome affect fertility or future reproductive health?
There is no direct evidence that it affects fertility. However, children receiving intensive treatments like chemotherapy should discuss this with their doctor.
Can the disease go away on its own without treatment?
No, Castleman Syndrome does not resolve by itself. Treatment and follow-up are necessary to prevent serious complications such as severe immune deficiency or organ failure.
Are there new treatments or research?
Yes, recent research is exploring new immune-targeted therapies and biologics, showing promising results, especially for multicentric Castleman Disease (MCD).

Stages of Castleman Syndrome in Children
Castleman Syndrome does not progress the same way in all children, but general stages can be observed depending on the type (UCD or MCD):

1️⃣ Silent stage (no obvious symptoms)

Usually in unicentric type (UCD).
Child may have a small lymph node enlargement without symptoms.
Sometimes discovered by accident during imaging for another reason.

2️⃣ Early stage (mild symptoms)

Gradual enlargement of lymph nodes.
Mild general symptoms: slight fatigue, poor appetite, slow growth, mild headache or dizziness.
Often not noticeable to parents at first.

3️⃣ Intermediate stage (increased symptoms)

More common in multicentric type (MCD).
Lymph nodes enlarge in multiple areas.
Symptoms become more obvious:
- Recurrent fever
- Significant weight loss
- Enlarged spleen or liver
- Anemia (paleness + persistent fatigue)

4️⃣ Advanced stage (complications)

Occurs if untreated or diagnosed late.
Immune problems and increased infections.
Blood clots or bleeding due to blood disorders.
Organ failure (liver or kidneys).
In virus-associated types (e.g., HHV-8), complications can progress faster.

5️⃣ Chronic Stage (Long-Term)
This stage usually occurs with the multicentric type (MCD).
The disease can persist for a long time and requires ongoing monitoring.
Effects may include:

Slowed physical growth
Difficulty concentrating or academic delays
Weak immunity with recurrent infections

Causes of Castleman Syndrome in Children
Castleman Disease is very rare in children, and doctors have not identified a single clear cause. However, several factors may contribute:

1️⃣ Immune System Dysfunction
Sometimes the immune system is overactive or abnormal, causing lymph nodes to enlarge and produce symptoms.

2️⃣ Excess IL-6 Protein
IL-6 is a protein that activates the immune system. Excessive amounts can trigger inflammation and lymph node enlargement.

3️⃣ Viral Infections
Some studies link the syndrome to viruses such as human herpesvirus 8 (HHV-8), especially in children with weakened immunity.

4️⃣ Potential Genetic Factors
No conclusive evidence exists yet, but research suggests some children may have a genetic predisposition.

5️⃣ Unknown Causes (Idiopathic)
In many cases, the cause is unknown, classified as idiopathic Castleman Syndrome.

Symptoms of Castleman Syndrome in Children
Symptoms vary depending on the type: Unicentric (UCD) affects a single lymph node, while Multicentric (MCD) affects multiple areas.

Symptoms of Unicentric Castleman Syndrome (UCD):

Enlargement of a single lymph node (commonly in the chest, abdomen, or neck)
Child may show no symptoms; often discovered accidentally during exams
Sometimes pain or pressure at the swollen node

Symptoms of Multicentric Castleman Syndrome (MCD):

Widespread lymph node enlargement
Enlarged liver or spleen
Recurrent or persistent fever
Severe fatigue
Unexplained weight loss
Loss of appetite
Night sweats
Recurrent infections due to weakened immunity
Anemia causing paleness and dizziness

Types of Castleman Syndrome in Children
Castleman Disease has several types, affecting symptoms and treatment:

1️⃣ Unicentric (UCD)

Most common type in children
Affects a single lymph node or region
Often asymptomatic and discovered by chance
Treatment: usually surgical removal of the affected node, often leading to complete recovery

2️⃣ Multicentric (MCD)

Less common but more severe
Affects multiple lymph nodes and organs such as liver and spleen
Linked to excess IL-6 and immune dysfunction
Sometimes associated with HHV-8 or autoimmune conditions
Symptoms: fever, fatigue, weight loss, enlarged liver/spleen, anemia
Requires medication: immunosuppressants, IL-6 inhibitors, sometimes chemotherapy

3️⃣ HHV-8 Associated

Extremely rare in children, usually in immunocompromised cases
Caused by human herpesvirus 8, often linked to HIV

4️⃣ Idiopathic (iMCD)

Very rare in children
Cause unknown (not viral or genetic)
Similar symptoms to MCD, requires precise diagnosis

Complications of Castleman Syndrome in Children
Complications depend on the type and how quickly treatment is started:

Immune System

Weak immunity → frequent infections (lungs, digestive system)
Slower response to infections
Occasionally higher risk of autoimmune disorders or chronic inflammation

Blood & Circulation

Anemia → paleness, dizziness, persistent fatigue
Platelet disorders → recurrent bleeding
Blood clots in severe cases

Liver & Spleen

Enlarged liver or spleen → abdominal pain or swelling
Chronic cases: impaired liver or spleen function

Growth & Development

Loss of appetite and weight
Delayed physical growth
Reduced concentration and academic performance due to fatigue

Nervous System

Frequent headaches
Dizziness or fainting
Rarely: memory problems or neuropathy due to poor circulation

Severe Complications (Untreated Cases)

MCD: organ failure (liver, kidney, heart)
Increased risk of lymphoma
HHV-8 associated type: faster and more severe complications

Diagnosis of Castleman Syndrome in Children
Diagnosis is challenging because it can mimic other diseases like lymphoma or chronic infections. Steps include:

Medical History & Physical Exam

Ask parents about symptoms: recurrent fever, weight loss, night sweats, poor appetite, persistent fatigue
Check lymph nodes: single (UCD) or multiple (MCD)
Abdominal exam: enlarged liver or spleen

Blood Tests

Complete blood count (CBC) → detects anemia, low platelets, or inflammation
Liver and kidney function tests → assess organ involvement
CRP & ESR → usually elevated due to inflammation
Sometimes viral tests (HHV-8, HIV) if suspected

Imaging

CT scan → locate and size lymph nodes
MRI → detailed tissue imaging
Ultrasound → detect liver or spleen enlargement

Lymph Node Biopsy

Most important step for definitive diagnosis
Examined under a microscope to confirm Castleman Syndrome or rule out cancer

Additional Tests

Bone marrow exam → evaluate blood cell production
Immune and genetic tests → confirm syndrome type

Treatment of Castleman Syndrome in Children

1. Multicentric Castleman Disease (MCD)

Immunotherapy (Targeted Therapy):
- Siltuximab: IL-6 inhibitor, reduces inflammation and lymph node growth
- Tocilizumab: IL-6 receptor blocker, very effective
Chemotherapy:
- Cyclophosphamide or Vincristine for cases resistant to immunotherapy
Corticosteroids:
- Prednisone reduces inflammation and symptoms, not a definitive cure
Antivirals:
- If caused by HHV-8 or HIV

2. Unicentric Castleman Disease (UCD)

Usually treated with surgical removal of the affected node
Sometimes corticosteroids are used pre-surgery to shrink the node
Supportive medications if complications or surgical difficulty

3. Supportive Treatments

Antibiotics to control infections
Blood or platelet transfusions if severe deficiency
Pain relief and fever reducers

Vitamin & Nutritional Support
Vitamins are not a primary treatment but help strengthen immunity and reduce fatigue:

Vitamin C: strengthens immunity, improves iron absorption
- Sources: oranges, lemons, strawberries, tomatoes
Vitamin D: supports bone health and immunity, important for children on corticosteroids
- Sources: sunlight, eggs, fortified milk
Vitamin B12 & Folic Acid: essential for blood cell formation and anemia prevention
- Sources: liver, meat, spinach, legumes
Iron: prescribed if anemia due to iron deficiency
- Sources: red meat, lentils, spinach
Zinc: supports tissue repair and immunity
- Sources: nuts, legumes, dairy products

Tips for Parents:

Always consult the doctor before giving any supplements.
Natural food is the foundation; vitamins are just supportive.
Ensure your child drinks enough water to support circulation and reduce fatigue.
Avoid fast food and excessive sugar.

How to Manage Castleman Syndrome in Children
Castleman Syndrome is a rare disease that requires careful monitoring and special care at home. Parents play a crucial role in maintaining the child’s health and minimizing complications.

1. Regular Medical Follow-Up

Keep continuous follow-up with a pediatric hematologist or oncologist.
Stick to scheduled blood tests and imaging exams.
Do not stop any prescribed medication without consulting the doctor, even if the child improves.

2. Healthy Nutrition
Provide balanced meals including:

Proteins: meat, poultry, fish
Fresh vegetables and fruits rich in vitamins
Whole grains: oats, lentils, beans
Limit fast food and sugary foods
Ensure sufficient water intake throughout the day

3. Infection Prevention

Frequent handwashing for both the child and family members
Avoid contact with sick people, especially during colds or flu seasons
Consult the doctor about appropriate and safe vaccinations
Monitor any fever immediately, as it could indicate infection

4. Psychological Support

Encourage the child with positive words and activities they enjoy
Provide a calm and stable home environment to reduce stress
Help them cope with fear of hospitals or medications

5. Physical Activity

Encourage light physical activity or age-appropriate exercises like walking or simple games
Avoid strenuous activities if the child is tired or anemic
Ask the doctor before starting any intense exercise

6. Symptom Monitoring
Keep track of any changes such as: