Castleman Disease Website – Castleman Disease Website

UNDERSTANDING CASTLEMAN DISEASE
DIAGNOSING CASTLEMAN DISEASE
TREATING CASTLEMAN DISEASE

UNDERSTANDING CASTLEMAN DISEASE
DIAGNOSING CASTLEMAN DISEASE
TREATING CASTLEMAN DISEASE

UNDERSTANDING CASTLEMAN DISEASE
DIAGNOSING CASTLEMAN DISEASE
TREATING CASTLEMAN DISEASE

INTRODUCTION
SIGNS AND SYMPTOMS

INTRODUCTION
SIGNS AND SYMPTOMS

INTRODUCTION
PATIENT STORIES
iMCD BOOKLET

FINDING SUPPORT
DOWNLOADABLE RESOURCES
USEFUL TERMS

Castleman disease is a

involving enlarged lymph nodes^1,2

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.¹
Groups of lymph nodes are found in lots of different parts of your body.^1,2

In Castleman disease, lymph cells multiply more than normal, directed by hyperactive immune system signals.^1,2,5

This causes the lymph node to

get bigger

It also causes changes in the lymph node features^1,5

These can only be seen using a

microscope

Castleman disease behaves like cancers of the lymph nodes known as ^2,5

lymphoma

However, it is important to know that Castleman disease is not a cancer, and it is treated differently.^2,5 However, Castleman disease can increase the risk of developing some types of cancer.²

Anyone can develop

Castleman disease

– men, women, adults, or children^1,2

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.¹

Learn More

Multicentric Castleman disease (MCD)

When two or more groups of lymph nodes contain lymph nodes that are larger than normal and have microscopic changes.¹

Learn More

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. ²

Carry out a physical examination

Your doctor will check your lymph nodes, spleen and liver.² They may also check for signs of infection – this is because infection is the most common cause of large lymph nodes.²

You may be referred to specialist doctors

Depending on the symptoms you are experiencing, a specialist such as a haematologist, rheumatologist, or immunologist may be more familiar with diagnosing and treating this rare disease.⁴

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.^2,3

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.^2,3

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.^2,3

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.²

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.²

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.²

MCD is usually treated with medicines that target specific parts of your immune system

Your doctor will recommend a treatment depending on the type of MCD.^2,3

REFERENCES

References

Castleman Disease Collaborative Network: https://cdcn.org/castleman-disease/overview/ (Date Accessed May 2025)
National Organization for Rare Disorders: https://rarediseases.org/rare-diseases/castlemans-disease/ (Date Accessed May 2025)
van Rhee F et al. International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease. Blood 2018;132(20):2115–2124. https://doi.org/10.1182/blood-2018-07-862334
DukeHealth Referring Physicians: https://physicians.dukehealth.org/articles/castlemans-disease-presents-challenges-diagnosis-treatment (Date Accessed May 2025)
Dispenzieri A, Fajgenbaum DC. Overview of Castleman disease. Blood. 2020;135(16):1353–1364. https://doi.org/10.1182/blood.2019000931

SKIP

SKIP

SKIP

Castleman disease is a

involving enlarged lymph nodes^1,2

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.¹
Groups of lymph nodes are found in lots of different parts of your body.^1,2

In Castleman disease, lymph cells multiply more than normal, directed by hyperactive immune system signals.^1,2,5

This causes the lymph node to

get bigger

It also causes changes in the lymph node features^1,5

These can only be seen using a

microscope

Castleman disease behaves like cancers of the lymph nodes known as ^2,5

lymphoma

However, it is important to know that Castleman disease is not a cancer, and it is treated differently.^2,5 However, Castleman disease can increase the risk of developing some types of cancer.²

Anyone can develop

Castleman disease

– men, women, adults, or children^1,2

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.¹

Learn More

Multicentric Castleman disease (MCD)

When two or more groups of lymph nodes contain lymph nodes that are larger than normal and have microscopic changes.¹

Learn More

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. ²

Carry out a physical examination

Your doctor will check your lymph nodes, spleen and liver.² They may also check for signs of infection – this is because infection is the most common cause of large lymph nodes.²

You may be referred to specialist doctors

Depending on the symptoms you are experiencing, a specialist such as a haematologist, rheumatologist, or immunologist may be more familiar with diagnosing and treating this rare disease.⁴

Order blood and imaging tests

Recommend a lymph node biopsy

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.²

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.²

MCD is usually treated with medicines that target specific parts of your immune system

Your doctor will recommend a treatment depending on the type of MCD.^2,3

REFERENCES

References

Castleman Disease Collaborative Network: https://cdcn.org/castleman-disease/overview/ (Date Accessed May 2025)
National Organization for Rare Disorders: https://rarediseases.org/rare-diseases/castlemans-disease/ (Date Accessed May 2025)
van Rhee F et al. International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease. Blood 2018;132(20):2115–2124. https://doi.org/10.1182/blood-2018-07-862334
DukeHealth Referring Physicians: https://physicians.dukehealth.org/articles/castlemans-disease-presents-challenges-diagnosis-treatment (Date Accessed May 2025)
Dispenzieri A, Fajgenbaum DC. Overview of Castleman disease. Blood. 2020;135(16):1353–1364. https://doi.org/10.1182/blood.2019000931

Castleman disease is a

involving enlarged lymph nodes^1,2

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.¹
Groups of lymph nodes are found in lots of different parts of your body.^1,2

In Castleman disease, lymph cells multiply more than normal, directed by hyperactive immune system signals.^1,2,5

This causes the lymph node to

get bigger

It also causes changes in the lymph node features^1,5

These can only be seen using a

microscope

Castleman disease behaves like cancers of the lymph nodes known as ^2,5

lymphoma

However, it is important to know that Castleman disease is not a cancer, and it is treated differently.^2,5 However, Castleman disease can increase the risk of developing some types of cancer.²

Anyone can develop

Castleman disease

– men, women, adults, or children^1,2

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.¹

Learn More

Multicentric Castleman disease (MCD)

When two or more groups of lymph nodes contain lymph nodes that are larger than normal and have microscopic changes.¹

Learn More

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. ²

Carry out a physical examination

Your doctor will check your lymph nodes, spleen and liver.² They may also check for signs of infection – this is because infection is the most common cause of large lymph nodes.²

You may be referred to specialist doctors

Depending on the symptoms you are experiencing, a specialist such as a haematologist, rheumatologist, or immunologist may be more familiar with diagnosing and treating this rare disease.⁴

Order blood and imaging tests

Recommend a lymph node biopsy

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.²

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.²

MCD is usually treated with medicines that target specific parts of your immune system

Your doctor will recommend a treatment depending on the type of MCD.^2,3

REFERENCES

References

Castleman Disease Collaborative Network: https://cdcn.org/castleman-disease/overview/ (Date Accessed May 2025)
National Organization for Rare Disorders: https://rarediseases.org/rare-diseases/castlemans-disease/ (Date Accessed May 2025)
van Rhee F et al. International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease. Blood 2018;132(20):2115–2124. https://doi.org/10.1182/blood-2018-07-862334
DukeHealth Referring Physicians: https://physicians.dukehealth.org/articles/castlemans-disease-presents-challenges-diagnosis-treatment (Date Accessed May 2025)
Dispenzieri A, Fajgenbaum DC. Overview of Castleman disease. Blood. 2020;135(16):1353–1364. https://doi.org/10.1182/blood.2019000931

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Welcome to the Castleman

Patient Hub

Lymph nodes

Lymph nodes are part of your immune system.

This causes the lymph node to

get bigger

These can only be seen using a

microscope

Castleman disease behaves like cancers of the lymph nodes known as 2,5

lymphoma

Anyone can develop

Castleman disease

– men, women, adults, or children1,2

There are

two main types

of Castleman disease

Unicentric Castleman disease (UCD)

Multicentric Castleman disease (MCD)

How is Castleman disease

diagnosed?

Ask about your symptoms and your medical history

Carry out a physical examination

You may be referred to specialist doctors

Order blood and imaging tests

Recommend a lymph node biopsy

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

How is Castleman disease

treated?

UCD is usually treated by removing the affected lymph node surgically

MCD is usually treated with medicines that target specific parts of your immune system

Share your

Castleman story

Lymph nodes

Lymph nodes are part of your immune system.

This causes the lymph node to

get bigger

These can only be seen using a

microscope

Castleman disease behaves like cancers of the lymph nodes known as 2,5

lymphoma

Anyone can develop

Castleman disease

– men, women, adults, or children1,2

There are

two main types

of Castleman disease

Unicentric Castleman disease (UCD)

Multicentric Castleman disease (MCD)

How is Castleman disease

diagnosed?

Ask about your symptoms and your medical history

Carry out a physical examination

You may be referred to specialist doctors

Order blood and imaging tests

Recommend a lymph node biopsy

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

How is Castleman disease

treated?

UCD is usually treated by removing the affected lymph node surgically

MCD is usually treated with medicines that target specific parts of your immune system

Share your

Castleman story

Lymph nodes

Lymph nodes are part of your immune system.

This causes the lymph node to

get bigger

These can only be seen using a

microscope

Castleman disease behaves like cancers of the lymph nodes known as 2,5

lymphoma

Anyone can develop

Castleman disease

– men, women, adults, or children1,2

There are

two main types

of Castleman disease

Unicentric Castleman disease (UCD)

Multicentric Castleman disease (MCD)

How is Castleman disease

diagnosed?

Castleman disease behaves like cancers of the lymph nodes known as ^2,5

– men, women, adults, or children^1,2

UCD is usually treated by
removing the affected lymph
node surgically

Castleman disease behaves like cancers of the lymph nodes known as ^2,5

– men, women, adults, or children^1,2

UCD is usually treated by
removing the affected lymph
node surgically

Castleman disease behaves like cancers of the lymph nodes known as ^2,5

– men, women, adults, or children^1,2

UCD is usually treated by
removing the affected lymph
node surgically