Castleman Disease Website – Castleman Disease Website

UNDERSTANDING CASTLEMAN DISEASE
DIAGNOSING CASTLEMAN DISEASE
TREATING CASTLEMAN DISEASE
SHARE YOUR STORY

UNDERSTANDING CASTLEMAN DISEASE
DIAGNOSING CASTLEMAN DISEASE
TREATING CASTLEMAN DISEASE
SHARE YOUR STORY

UNDERSTANDING CASTLEMAN DISEASE
DIAGNOSING CASTLEMAN DISEASE
TREATING CASTLEMAN DISEASE
SHARE YOUR STORY

INTRODUCTION
SIGNS AND SYMPTOMS

INTRODUCTION
SIGNS AND SYMPTOMS

INTRODUCTION
PATIENT STORIES
SHARE YOUR STORY

FINDING SUPPORT
PATIENT ORGANISATIONS
DOWNLOADABLE RESOURCES
USEFUL TERMS

Castleman disease is a

involving abnormal lymph nodes

Scroll to continue

Lymph nodes

Lymph nodes are part of your immune system.

They contain specific types of cells which help your body fight off infections.
Groups of lymph nodes are found in lots of different parts of your body.

In Castleman disease, the number of cells in a lymph node increase because the cells multiply more than normal.

This causes the lymph node to

get bigger

It also causes changes in the lymph node structure

These can only be seen using a

microscope

Castleman disease behaves like cancers of the lymph nodes known as

lymphoma

However, it is important to know that Castleman disease is not a cancer, and it is treated differently. However, Castleman disease can increase the risk of developing some types of cancer.

Anyone can develop

Castleman disease

– men, women, adults, or children

LYMPH NODE

CELLS

There are

two main types

of Castleman disease

Unicentric Castleman disease (UCD)

When one group of lymph nodes has one or more lymph nodes that are larger than normal and have microscopic changes.

Multicentric Castleman disease (MCD)

When two or more groups of lymph nodes contain lymph nodes that are larger than normal and have microscopic changes.

How is Castleman disease

diagnosed?

To correctly diagnose Castleman disease your doctor will:

Ask about your symptoms and your medical history

Tell your doctor about your symptoms and when they started. Also tell them about any other diseases or medical conditions you have or have had in the past.

Carry out a physical examination

Your doctor will check your lymph nodes, spleen and liver. They may also check for signs of infection – this is because infection is the most common cause of large lymph nodes.

You may be referred to specialist doctors

Depending on the symptoms you are experiencing, a specialist such as a haematologist, rheumatologist, or infectiologist may be more familiar with diagnosing and treating this rare disease.

Order blood and imaging tests

Your doctor will order blood tests. Lots of information can be obtained from a blood test to help with the diagnosis. Your doctor will look for specific substances in your blood which can be higher or lower in patients with Castleman disease.

Your doctor will also send you for imaging tests, such as X-rays or scans, which will show any swollen or large lymph nodes or other problems that may be causing your symptoms.

Recommend a lymph node biopsy

Your doctor will review your symptoms, examination, and test results. While ruling out other diseases, your doctor may recommend you have a lymph node biopsy to confirm the diagnosis.

A lymph node biopsy is when all or part of the affected lymph node is removed in a minor operation. A microscope is then used to examine the lymph node to determine if there are changes that are commonly seen in Castleman disease.

It may take your doctor a while to correctly diagnose Castleman disease

This is because it is a very rare disease, and the symptoms are similar to those that occur with other diseases.

How is Castleman disease

treated?

UCD is usually treated by
removing the affected lymph
node surgically

Additional treatments, such as radiotherapy or medications, may be used if the operation is unsuccessful or not possible.

MCD treatments usually
target specific parts of your
immune system

Your doctor will recommend a treatment depending on the type of MCD.

The

treatments for MCD

can include:¹

Immunotherapy

An infused medicine that targets the immune system and is used as the first treatment for people with MCD. It is often life-long treatment.

Corticosteroids

These medicines reduce the activity of the immune system and are sometimes given on their own or with immune or other therapies.

Chemotherapy

This type of medicine shrinks the fast-growing cells in lymph nodes and is given to people with severe MCD or if the first treatments are not effective.

Antiviral therapy

Used to treat people with HHV8-positive MCD or to manage infections that sometimes appear in people with iMCD.

REFERENCES

References

van Rhee F et al. International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease. Blood 2018;132(20):2115–2124.

Sources:
American Cancer Society: https://www.cancer.org/cancer/castleman-disease (Date Accessed 6 Nov 2023)
Genetic and Rare Diseases Information Centre, Multicentric Castleman disease: https://rarediseases.info.nih.gov/diseases/9644/multicentric-castleman-disease (Date Accessed 6 Nov 2023)
National Organization for Rare Disorders: https://rarediseases.org/rare-diseases/castlemans-disease/ (Date Accessed 6 Nov 2023)

SKIP

SKIP

SKIP

Castleman disease is a

involving abnormal lymph nodes

Scroll to continue

Lymph nodes

Lymph nodes are part of your immune system.

They contain specific types of cells which help your body fight off infections.
Groups of lymph nodes are found in lots of different parts of your body.

In Castleman disease, the number of cells in a lymph node increase because the cells multiply more than normal.

This causes the lymph node to

get bigger

It also causes changes in the lymph node structure

These can only be seen using a

microscope

Castleman disease behaves like cancers of the lymph nodes known as

lymphoma

However, it is important to know that Castleman disease is not a cancer, and it is treated differently. However, Castleman disease can increase the risk of developing some types of cancer.

Anyone can develop

Castleman disease

– men, women, adults, or children

LYMPH NODE

CELLS

There are

two main types

of Castleman disease

Unicentric Castleman disease (UCD)

When one group of lymph nodes has one or more lymph nodes that are larger than normal and have microscopic changes.

Multicentric Castleman disease (MCD)

When two or more groups of lymph nodes contain lymph nodes that are larger than normal and have microscopic changes.

How is Castleman disease

diagnosed?

To correctly diagnose Castleman disease your doctor will:

Ask about your symptoms and your medical history

Tell your doctor about your symptoms and when they started. Also tell them about any other diseases or medical conditions you have or have had in the past.

Carry out a physical examination

Your doctor will check your lymph nodes, spleen and liver. They may also check for signs of infection – this is because infection is the most common cause of large lymph nodes.

You may be referred to specialist doctors

Depending on the symptoms you are experiencing, a specialist such as a haematologist, rheumatologist, or infectiologist may be more familiar with diagnosing and treating this rare disease.

Order blood and imaging tests

Your doctor will order blood tests. Lots of information can be obtained from a blood test to help with the diagnosis. Your doctor will look for specific substances in your blood which can be higher or lower in patients with Castleman disease.

Your doctor will also send you for imaging tests, such as X-rays or scans, which will show any swollen or large lymph nodes or other problems that may be causing your symptoms.

Recommend a lymph node biopsy

Your doctor will review your symptoms, examination, and test results. While ruling out other diseases, your doctor may recommend you have a lymph node biopsy to confirm the diagnosis.

A lymph node biopsy is when all or part of the affected lymph node is removed in a minor operation. A microscope is then used to examine the lymph node to determine if there are changes that are commonly seen in Castleman disease.

It may take your doctor a while to correctly diagnose Castleman disease

This is because it is a very rare disease, and the symptoms are similar to those that occur with other diseases.

How is Castleman disease

treated?

UCD is usually treated by
removing the affected lymph
node surgically

Additional treatments, such as radiotherapy or medications, may be used if the operation is unsuccessful or not possible.

MCD treatments usually
target specific parts of your
immune system

Your doctor will recommend a treatment depending on the type of MCD.

The

treatments for MCD

can include:¹

Immunotherapy

An infused medicine that targets the immune system and is used as the first treatment for people with MCD. It is often life-long treatment.

Corticosteroids

These medicines reduce the activity of the immune system and are sometimes given on their own or with immune or other therapies.

Chemotherapy

This type of medicine shrinks the fast-growing cells in lymph nodes and is given to people with severe MCD or if the first treatments are not effective.

Antiviral therapy

Used to treat people with HHV8-positive MCD or to manage infections that sometimes appear in people with iMCD.

REFERENCES

References

van Rhee F et al. International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease. Blood 2018;132(20):2115–2124.

Sources:
American Cancer Society: https://www.cancer.org/cancer/castleman-disease (Date Accessed 6 Nov 2023)
Genetic and Rare Diseases Information Centre, Multicentric Castleman disease: https://rarediseases.info.nih.gov/diseases/9644/multicentric-castleman-disease (Date Accessed 6 Nov 2023)
National Organization for Rare Disorders: https://rarediseases.org/rare-diseases/castlemans-disease/ (Date Accessed 6 Nov 2023)

Castleman disease is a

involving abnormal lymph nodes

Scroll to continue

Lymph nodes

Lymph nodes are part of your immune system.

They contain specific types of cells which help your body fight off infections.
Groups of lymph nodes are found in lots of different parts of your body.

In Castleman disease, the number of cells in a lymph node increase because the cells multiply more than normal.

This causes the lymph node to

get bigger

It also causes changes in the lymph node structure

These can only be seen using a

microscope

Castleman disease behaves like cancers of the lymph nodes known as

lymphoma

However, it is important to know that Castleman disease is not a cancer, and it is treated differently. However, Castleman disease can increase the risk of developing some types of cancer.

Anyone can develop

Castleman disease

– men, women, adults, or children

LYMPH NODE

CELLS

There are

two main types

of Castleman disease

Unicentric Castleman disease (UCD)

When one group of lymph nodes has one or more lymph nodes that are larger than normal and have microscopic changes.

Multicentric Castleman disease (MCD)

When two or more groups of lymph nodes contain lymph nodes that are larger than normal and have microscopic changes.

How is Castleman disease

diagnosed?

To correctly diagnose Castleman disease your doctor will:

Ask about your symptoms and your medical history

Tell your doctor about your symptoms and when they started. Also tell them about any other diseases or medical conditions you have or have had in the past.

Carry out a physical examination

Your doctor will check your lymph nodes, spleen and liver. They may also check for signs of infection – this is because infection is the most common cause of large lymph nodes.

You may be referred to specialist doctors

Depending on the symptoms you are experiencing, a specialist such as a haematologist, rheumatologist, or infectiologist may be more familiar with diagnosing and treating this rare disease.

Order blood and imaging tests

Your doctor will order blood tests. Lots of information can be obtained from a blood test to help with the diagnosis. Your doctor will look for specific substances in your blood which can be higher or lower in patients with Castleman disease.

Your doctor will also send you for imaging tests, such as X-rays or scans, which will show any swollen or large lymph nodes or other problems that may be causing your symptoms.

Recommend a lymph node biopsy

Your doctor will review your symptoms, examination, and test results. While ruling out other diseases, your doctor may recommend you have a lymph node biopsy to confirm the diagnosis.

A lymph node biopsy is when all or part of the affected lymph node is removed in a minor operation. A microscope is then used to examine the lymph node to determine if there are changes that are commonly seen in Castleman disease.

It may take your doctor a while to correctly diagnose Castleman disease

This is because it is a very rare disease, and the symptoms are similar to those that occur with other diseases.

How is Castleman disease

treated?

UCD is usually treated by
removing the affected lymph
node surgically

Additional treatments, such as radiotherapy or medications, may be used if the operation is unsuccessful or not possible.

MCD treatments usually
target specific parts of your
immune system

Your doctor will recommend a treatment depending on the type of MCD.

The

treatments for MCD

can include:¹

Immunotherapy

An infused medicine that targets the immune system and is used as the first treatment for people with MCD. It is often life-long treatment.

Corticosteroids

These medicines reduce the activity of the immune system and are sometimes given on their own or with immune or other therapies.

Chemotherapy

This type of medicine shrinks the fast-growing cells in lymph nodes and is given to people with severe MCD or if the first treatments are not effective.

Antiviral therapy

Used to treat people with HHV8-positive MCD or to manage infections that sometimes appear in people with iMCD.

REFERENCES

References

van Rhee F et al. International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease. Blood 2018;132(20):2115–2124.

Sources:
American Cancer Society: https://www.cancer.org/cancer/castleman-disease (Date Accessed 6 Nov 2023)
Genetic and Rare Diseases Information Centre, Multicentric Castleman disease: https://rarediseases.info.nih.gov/diseases/9644/multicentric-castleman-disease (Date Accessed 6 Nov 2023)
National Organization for Rare Disorders: https://rarediseases.org/rare-diseases/castlemans-disease/ (Date Accessed 6 Nov 2023)

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