Castleman Syndrome in Children Diagnosis Complications and Treatment Options

Many parents are often surprised after the birth of their child by swollen lymph nodes or other unusual symptoms, which could be an indicator of Castleman syndrome in children. This condition is rare but very important, as it can affect the child’s growth, body functions, and sometimes cause serious health complications if not diagnosed and treated early.In this article from Dlely Medical, we will explore everything about Castleman syndrome in children, including:

The causes of the syndrome and its types
Key symptoms to watch for
Accurate diagnostic methods
The different stages of the condition
Treatment options, both medication and surgery
Essential tips for parents to monitor their child with doctors and ensure healthy and safe growth

Castleman Syndrome in Children: Causes, Symptoms, Diagnosis, and Treatment

1️⃣ What is Castleman Syndrome in Children?

Castleman syndrome is a rare condition that affects the lymphatic system in children. A child may show swollen lymph nodes and sometimes enlargement of the spleen or liver. If not properly monitored, this condition can affect the child’s body functions.

2️⃣ Causes of Castleman Syndrome in Children

In most cases, the exact cause is unknown, but it can sometimes be associated with:

Viral infections, such as HHV-8 virus.
Immune system dysfunction.
Overproduction of certain inflammatory proteins in the body.

3️⃣ Symptoms of Castleman Syndrome in Children

Swollen lymph nodes in the neck, armpits, or abdomen.
Persistent or recurring fever.
Weight loss or general weakness.
Sometimes enlarged spleen or liver.
Severe fatigue or night sweats.

4️⃣ Is Castleman Syndrome Dangerous?

Unicentric Castleman Disease (UCD): Usually less severe and can be treated surgically.
Multicentric Castleman Disease (MCD): More complex, requires continuous monitoring and careful treatment to avoid complications in the heart, liver, or kidneys.

5️⃣ How is Castleman Syndrome Diagnosed in Children?

Clinical examination: To check for swollen lymph nodes and affected organs.
Blood tests: To detect inflammation or immune system problems.
Imaging tests: CT scan or MRI to determine the size and location of lymph nodes.
Lymph node biopsy: Sometimes needed to confirm the diagnosis.

6️⃣ Treatment of Castleman Syndrome in Children

Medication: Anti-inflammatory drugs, sometimes immunosuppressants or targeted biological therapy depending on the type.
Surgery: Removal of enlarged lymph nodes or associated tumors if they cause problems.
Regular follow-up: Essential to monitor the child’s growth, organ function, and prevent complications.

Stages of Castleman Syndrome in Children

1️⃣ Early Stage

Limited swelling in one or two lymph nodes.
The child may have no symptoms or mild fatigue.
Often discovered incidentally during routine check-ups.

2️⃣ Progressive Stage

Lymph nodes enlarge and may extend to multiple areas.
General symptoms appear: intermittent fever, weight loss, night sweats, persistent fatigue.
Sometimes enlarged liver or spleen (hepatosplenomegaly).

3️⃣ Severe / Multicentric Stage

Lymph nodes enlarged in multiple regions of the body.
Additional problems: pressure on internal organs, anemia, or low platelets.
Some children may require urgent medical intervention or intensive medication.

Causes of Castleman Syndrome in Children

While the direct cause is not fully known, doctors have linked it to:

1️⃣ Immune system dysfunction

Overactive B and T cells in some cases.
Leads to excessive production of inflammatory substances like interleukin-6 (IL-6) → causing lymph node swelling and other symptoms.

2️⃣ Viral factors

In Multicentric Castleman Disease (MCD), sometimes associated with HHV-8 virus.
The virus continuously stimulates the immune system, though it is less common in children than adults.

3️⃣ Genetic / hereditary factors (rare)

No specific gene identified, but studies suggest some children may have a genetic predisposition.

4️⃣ Coexisting diseases or environmental factors

Weak immunity or exposure to certain infections may increase the risk.

Key Symptoms of Castleman Syndrome in Children

Lymphadenopathy: Swelling of lymph nodes in one (UCD) or multiple areas (MCD). May cause pain or pressure on nearby organs.
Fever and persistent fatigue: Repeated or chronic fever without clear cause.
Weight loss and poor appetite: Rapid weight loss due to inflammation or low appetite.
Hepatosplenomegaly: Especially in MCD, can cause abdominal pain or swelling.
Blood problems:
- Low red blood cells → anemia.
- Low platelets → easy bleeding or bruising.
Other systemic symptoms: Night sweats, swelling of limbs or face, occasional mild rash.
Rare complications: Kidney problems or high blood pressure from chronic inflammation; immune disorders making the child more susceptible to infections.

Types of Castleman Syndrome in Children

1️⃣ Unicentric Castleman Disease (UCD)

Single lymph node or small group of nodes in one area.
Most children have no major systemic symptoms.
May cause pressure on nearby organs depending on location.
Treatment: Usually surgical removal of the node → often full recovery.

Multicentric Castleman Disease (MCD) in Children, Complications, Diagnosis, Treatment, and Nutrition

1️⃣ Multicentric Castleman Disease (MCD)

Multiple lymph nodes affected in different areas of the body.
Systemic symptoms: recurrent fever, weight loss, fatigue, enlarged liver and spleen.
Often associated with immune dysfunction or viral infection (sometimes HHV-8).
Treatment: requires immunosuppressive or chemotherapy medications with long-term follow-up; surgery alone is not sufficient if the disease is widespread.

2️⃣ Complications and Diagnosis in Children

Complications by Type

1️⃣ Unicentric Castleman Disease (UCD)

Usually involves a single lymph node in a specific location → complications are minimal.
Possible issues:
- Pressure on nearby organs: lungs, stomach, or nerves → may cause breathing or swallowing difficulties.
- Inflammation or infection if the node is affected.
After surgical removal, most children recover fully.

2️⃣ Multicentric Castleman Disease (MCD)

More severe because lymph nodes are widespread and the immune system reacts strongly.
Possible problems:
- Enlarged liver and spleen → organ function issues.
- Anemia or low platelets → fatigue and bleeding tendency.
- Frequent infections due to weakened immunity.
- Chronic inflammation → continuous fever, severe fatigue, weight loss.
- Rarely, increased risk of blood cancers or lymphoma.

Diagnosis in Children

1️⃣ Clinical examination

Observe swollen lymph nodes or abnormal masses.
Evaluate associated symptoms: fever, weight loss, fatigue, or liver/spleen enlargement.

2️⃣ Laboratory tests

Complete blood count (CBC): detect anemia or low platelets.
Inflammatory markers: CRP, ESR for chronic inflammation.
Liver and kidney function tests if organs are affected.

3️⃣ Imaging tests

Ultrasound: locate and measure lymph nodes or affected organs.
MRI or CT scan: evaluate the extent and size of lymph node involvement.

4️⃣ Biopsy

Take a sample of the affected lymph node for microscopic analysis.
Confirms diagnosis and rules out other tumors or similar diseases.

5️⃣ Differential diagnosis

Exclude other causes of lymph node enlargement:
- Chronic bacterial or viral infections
- Lymphoma or other tumors
- Rare immune disorders

⚠️ Risks and Complications

1️⃣ Problems due to enlarged lymph nodes

Pressure on lungs, stomach, or intestines → difficulty breathing or swallowing.
Abdominal pain or discomfort.

2️⃣ Blood problems

Anemia: fatigue and general weakness.
Thrombocytopenia: easy bleeding or bruising.
Sometimes immune disorders due to lymph node dysfunction.

3️⃣ Systemic issues

Recurrent fever or night sweats.
Weight loss or poor growth in advanced cases.

4️⃣ Cancer risk

Some forms of MCD may increase the likelihood of:
- Lymph node tumors
- Long-term blood or immune system problems

5️⃣ Psychological and social impact

Visible lymph node enlargement may cause stress or social embarrassment.

3️⃣ Treatment of Castleman Syndrome in Children

1️⃣ Medication

A. Anti-inflammatory and immunosuppressive drugs

Corticosteroids: reduce inflammation and lymph node swelling.
Immunosuppressants: e.g., cyclosporine or tacrolimus, especially for severe or multicentric cases.

B. Targeted biological therapy

New drugs targeting specific proteins like IL-6 inhibitors (e.g., Siltuximab or Tocilizumab).
Particularly useful for widespread or chronic cases.

C. Blood-supporting medications

Blood or platelet transfusions if needed.
Sometimes drugs to stimulate blood cell production.

D. Symptom management

Medications for fever or pain.
Treat liver or kidney problems if affected.

2️⃣ Surgery

Depends on type and size of lymph node or organ enlargement.

A. Removal of enlarged lymph nodes

If nodes press on vital organs → surgical removal may be needed.
Mainly for UCD, as MCD is usually too widespread for complete removal.

B. Correcting affected organs

Surgery may relieve pressure on liver, spleen, or kidneys.
Sometimes partial removal of spleen or liver is needed.

C. Removal of associated tumors or growths

Some children may develop benign or small tumors → surgery prevents future complications.

D. Important notes

Surgery is usually optional or for specific cases.
Decision depends on node size, organ impact, and overall child health.
Post-surgery follow-up is essential to prevent recurrence or new problems.

4️⃣ Tips for Parents to Monitor Their Child

1️⃣ Regular medical follow-up

Schedule visits according to doctor’s instructions.
Track symptom progression and treatment response.

2️⃣ Daily symptom monitoring

Watch for new lymph node swelling.
Record fever, fatigue, weight loss, or bruising.
Report sudden changes immediately.

3️⃣ Follow treatment plan carefully

Adhere to prescribed medications: corticosteroids, immunosuppressants, or biologics.
Keep transfusion schedules if needed.

4️⃣ Healthy and supportive nutrition

Balanced meals to support immunity and growth.
Adequate water intake to protect kidneys and liver.

5️⃣ Monitor growth and development

Track weight, height, and cognitive/physical development.
Note any delays or behavioral changes.

6️⃣ Psychological and social support

Encourage social interaction despite visible lymph node swelling.
Provide emotional support to prevent stress or embarrassment.

7️⃣ Maintain a complete medical record

Keep track of all tests, scans, and medication schedules.
Helps the doctor assess condition and adjust treatment.

8️⃣ Manage emergencies

Seek immediate care if bleeding, breathing difficulty, severe pain, or new swelling occurs.

5️⃣ Nutrition for Children with Castleman Syndrome

Balanced meals: fruits, vegetables, proteins (meat, poultry, fish, eggs), whole grains.
Adequate fluids: water, natural juices to boost vitamin C and immunity.
Blood and immunity support: iron-rich foods (red meat, lentils, spinach) and vitamins/minerals (A, C, D, calcium).
Small, frequent meals: 5–6 meals/day to reduce fatigue and aid digestion.
Avoid: high sugar, saturated fats, and processed foods.
Consult a pediatric nutritionist if appetite or weight is low → personalized diet plan supporting treatment.