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Unicentric Castleman Disease

Table of Contents

Unicentric Castleman Disease

UCD; Unicentric CD; Unicentric angiofollicular ganglionic hyperplasia; Unicentric angiofollicular lymph hyperplasia; Localized Castleman disease

Unicentric Castleman disease (UCD) is a rare condition that affects the lymph nodes and related tissues. It is a form of Castleman disease that is "localized" to a single set of lymph nodes.

Lymph Node and Lymph interstitial fluid

Image by TheVisualMD

Summary

Lymphatic System and Immune System

Image by TheVisualMD

Lymphatic System and Immune System

Our immune system is designed to react to anything foreign. When a pathogen enters our body, a system of special cells mobilizes in a coordinated defense response. This can involve the secretion of specific molecules to bind to and neutralize the pathogen or it can involve special cells that engulf or otherwise eliminate the germ. The immune system uses the bloodstream, the gastrointestinal tract, and the lymphatic system, which consists of a network of vessels that collect and carry a clear fluid called lymph that plays roles in immune function as well as the transport of nutrients and eventually empties into the bloodstream.

Image by TheVisualMD

What Is Unicentric Castleman Disease?

Unicentric Castleman disease (UCD) is a rare condition that affects the lymph nodes and related tissues. It is a form of Castleman disease that is "localized" to a single set of lymph nodes (as opposed to multicentric Castleman disease which has more widespread effects). UCD often starts as an enlarged lymph node. Depending on the location of this enlarged node, some people will have no additional features of the condition, while others will develop symptoms when it pushes on nearby organs and/or tissues. The exact underlying cause of UCD is currently unknown. Treatment typically includes surgical removal of the affected lymph node.

Source: Genetic and Rare Diseases (GARD) Information Center

Additional Materials (13)

Lymphatic, circulatory and nervous systems

The systems of the human body consist of: circulatory, digestive, endocrine, immune/lymphatic, integumentary, muscular, reproductive, skeletal, urinary. This image depicts the circulatory, endocrine, immune/lymphatic, nervous

Image by TheVisualMD

Castleman Disease

Castleman disease Preoperative CT scan : A)Castleman's disease Preoperative CT scan of the abdomen showing a highly vascularized retroperitoneal tumor measuring 10 × 9.2 cm with intratumor calcifications. Right ureter dilatation (grade II), (coronal multiplanar reformation, MPR) B) The same tumor in axial orientation. No other tumor localization in the abdomen nor enlarged lymph nodes were detected.

Image by Menenakos C, Braumann C, Hartmann J, Jacobi CA.

Diagnosis of Multicentric Castleman’s Disease

Video by Targeted Oncology/YouTube

What is Castleman Disease? Symptoms and Treatment | The Balancing Act

Video by The Balancing Act/YouTube

Castleman Disease Explained

Video by CheckRare/YouTube

Castleman Disease – A Pathology Perspective

Video by ASCP/YouTube

Castleman Disease: What You Need to Know

Video by Medical Centric/YouTube

Faces of Castleman Disease: Joey's Story

Video by Castleman Disease Collaborative Network/YouTube

Faces of Castleman Disease: Katie's Story

Video by Castleman Disease Collaborative Network/YouTube

Faces of Castleman Disease: Gary's Story

Video by Castleman Disease Collaborative Network/YouTube

Castleman's Disease - Signs and Symptoms

Video by Rare Disease Report/YouTube

Treatment Options for Castleman's Disease

Video by Rare Disease Report/YouTube

Unicentric Castleman’s Disease

Video by Targeted Oncology/YouTube

Lymphatic, circulatory and nervous systems

TheVisualMD

Castleman Disease

Menenakos C, Braumann C, Hartmann J, Jacobi CA.

4:56

Diagnosis of Multicentric Castleman’s Disease

Targeted Oncology/YouTube

8:34

What is Castleman Disease? Symptoms and Treatment | The Balancing Act

The Balancing Act/YouTube

2:03

Castleman Disease Explained

CheckRare/YouTube

18:39

Castleman Disease – A Pathology Perspective

ASCP/YouTube

3:53

Castleman Disease: What You Need to Know

Medical Centric/YouTube

3:40

Faces of Castleman Disease: Joey's Story

Castleman Disease Collaborative Network/YouTube

2:23

Faces of Castleman Disease: Katie's Story

Castleman Disease Collaborative Network/YouTube

3:23

Faces of Castleman Disease: Gary's Story

Castleman Disease Collaborative Network/YouTube

4:16

Castleman's Disease - Signs and Symptoms

Rare Disease Report/YouTube

6:06

Treatment Options for Castleman's Disease

Rare Disease Report/YouTube

6:13

Unicentric Castleman’s Disease

Targeted Oncology/YouTube

Symptoms

Difficulty Breathing

Image by AomAm/Noun Project

Difficulty Breathing

Image by AomAm/Noun Project

What Are the Signs and Symptoms of Unicentric Castleman Disease?

A feeling of fullness or pressure in the chest or abdomen
Trouble eating or breathing
Unintended weightloss
Less commonly, fever, night sweats and weakness

Source: Genetic and Rare Diseases (GARD) Information Center

Cause

IL6 Crystal Structure.rsh

Image by Ramin Herati/Wikimedia

IL6 Crystal Structure.rsh

Crystal structure of IL-6 as published in the Protein Data Bank (PDB: 1ALU)

Image by Ramin Herati/Wikimedia

What Causes Unicentric Castleman Disease?

The exact underlying cause of unicentric Castleman disease (UCD) is poorly understood. However, some scientists suspect that an increased production of interleukin-6 (IL-6) by the immune system may contribute to the development of UCD. IL-6 is a substance normally produced by cells within the lymph nodes that helps coordinate the immune response to infection. Increased production of IL-6 may result in an overgrowth of lymphatic cells, leading to many of the signs and symptoms of UCD.

Source: Genetic and Rare Diseases (GARD) Information Center

Inheritance

Idiopathic - Unknown

Image by PaliGraficas/Pixabay

Idiopathic - Unknown

Image by PaliGraficas/Pixabay

Is Unicentric Castleman Disease Inherited?

Although the exact underlying cause of unicentric Castleman disease (UCD) is unknown, it is thought to occur sporadically in people with no family history of the condition.

Source: Genetic and Rare Diseases (GARD) Information Center

Diagnosis

Blood Tests

Image by TheVisualMD

Blood Tests

Blood tests show whether the levels of different substances in your blood fall within a normal range. Some common blood tests are the complete blood count, which includes red blood cell, white blood cell, and platelet counts; the blood glucose test; and the lipoprotein panel, which measures cholesterol levels. Certain types of blood tests can help detect cancer, but additional tests are necessary to confirm diagnosis.

Image by TheVisualMD

How Is Unicentric Castleman Disease Diagnosed?

Blood tests can be ordered to evaluate the levels of Interleukin-6 (IL-6) and other substances in the body, which can be elevated in people with UCD. They can also be helpful in ruling out other autoimmune conditions and infections that are associated with similar signs and symptoms
Imaging studies (such as a CT scan, PET scan, MRI scan, and/or ultrasound) can help identify enlarged lymph node(s) and other health problems
Lymph node biopsy is usually recommended to confirm the diagnosis

Source: Genetic and Rare Diseases (GARD) Information Center

Interleukin-6 Test

Interleukin-6 Test

Also called: IL-6

Interleukin-6 (IL-6) is a protein that contributes to the immune system response against infections and tissue injuries; and it also plays a role in many other bodily functions, such as bone maintenance and body temperature regulation. IL-6 is primarily found during inflammatory processes and infections. This test measures the amount of IL-6 in the serum...

Interleukin-6 Test

Also called: IL-6

Interleukin-6 (IL-6) is a protein that contributes to the immune system response against infections and tissue injuries; and it also plays a role in many other bodily functions, such as bone maintenance and body temperature regulation. IL-6 is primarily found during inflammatory processes and infections. This test measures the amount of IL-6 in the serum...

{"label":"Interleukin-6 Reference Range","scale":"lin","step":0.1,"hideunits":false,"units":[{"printSymbol":"pg\/mL","code":"pg\/mL","name":"picogram per milliliter"}],"items":[{"flag":"normal","label":{"short":"Normal","long":"Normal","orientation":"horizontal"},"values":{"min":0,"max":15.5},"text":"Normal results can vary depending on the laboratory and the method used for testing. ","conditions":[]},{"flag":"abnormal","label":{"short":"High","long":"High","orientation":"horizontal"},"values":{"min":15.5,"max":40},"text":"Your interleukine-6 levels are increased. This usually indicates infection or inflammatory conditions.","conditions":["Rheumatoid arthritis","Systemic lupus erythematosus","Inflammatory bowel disease","Ankylosing spondylitis","Infections","Sepsis","Leukemia","Diabetes","Cardiovascular disease","Strenuous exercise"]}],"value":7.8}[{"normal":0},{"abnormal":0}]
Use the slider below to see how your results affect your health.
pg/mL
15.5
Your result is Normal.
Normal results can vary depending on the laboratory and the method used for testing.
Related conditions
In normal conditions, the IL-6 levels in the blood should be very low or undetectable. Nevertheless, a normal level does not rule out the possibility of an ongoing inflammatory process.
Increased IL-6 levels may indicate an inflammatory condition or an infection. It can be associated with:
- Rheumatoid arthritis
- Systemic lupus erythematosus
- Inflammatory bowel disease
- Ankylosing spondylitis
- Infections
- Sepsis
- Leukemia
- Diabetes
- Cardiovascular disease
- Strenuous exercise
IL-6 is also produced by fat cells (adipocytes); therefore, obese individuals have higher IL-6 levels.
1. 140916: Interleukin-6, Serum | LabCorp [accessed on Jan 05, 2019]
2. 140816: Interleukin-6, Plasma | LabCorp [accessed on Jan 05, 2019]
3. IL-6 in Inflammation, Immunity, and Disease [accessed on Jan 07, 2019]
4. Interleukin-6 [accessed on Jan 07, 2019]
5. IL6 - Clinical: Interleukin 6 (IL-6), Plasma [accessed on Jan 07, 2019]
6. Interleukin-6, Highly Sensitive, ELISA [accessed on Jan 07, 2019]

Normal reference ranges can vary depending on the laboratory and the method used for testing. You must use the range supplied by the laboratory that performed your test to evaluate whether your results are "within normal limits."

Treatment

Surgery

Image by Staff Sgt. Miguel Lara/Wikimedia

Surgery

U.S. Air Force Maj Jonathan Forbes, 455th Expeditionary Medical Operations Squadron neurosurgeon looks through loupes as he performs brain surgery at the Craig Joint Theater Hospital, Bagram Air Field, Afghanistan, Oct. 10, 2014. The loupes allow Forbes to see the surgery at a two times magnification. Forbes removed an 8-centimeter tumor during the five-hour surgery. Forbes is deployed from Travis Air Force Base, Calif., and is a native of New Martinsville, W.Va. (U.S. Air Force photo by Staff Sgt. Miguel Lara III/Released) Unit: 455th Air Expeditionary Wing

Image by Staff Sgt. Miguel Lara/Wikimedia

How Might Unicentric Castleman Disease Be Treated?

Source: Genetic and Rare Diseases (GARD) Information Center

Prognosis

Prognosis Icon

Image by mcmurryjulie/Pixabay

Prognosis Icon

Image by mcmurryjulie/Pixabay

What Is the Long-Term Outlook for People with Unicentric Castleman Disease?

The long-term outlook (prognosis) for people with unicentric Castleman disease (UCD) is generally excellent. In many cases, the condition can be cured by surgically removing the affected lymph node. In fact, one study found that disease-free survival in people with UCD following surgery was approximately 93%.

Source: Genetic and Rare Diseases (GARD) Information Center

Related HealthJournals

Castleman Disease

Castleman disease (CD) is a rare condition that affects the lymph nodes and related tissues. There are two main forms: unicentric CD and multicentric CD.

Multicentric Castleman Disease

Multicentric Castleman disease (MCD) is a rare disease that affects the lymph nodes and related tissues. It is a form of Castleman disease that is "systemic" and involves multiple sets of lymph nodes and other tissues throughout the body.

Lymphatic Diseases

Lymphatic diseases can affect your lymph, lymph vessels, or lymph nodes. Lymphatic system problems include lymphedema and swollen glands. Learn more.

Lymphatic Malformations

Lymphatic malformations or lymphangiomas are benign (non-cancerous) vascular lesions made of fluid-filled spaces (cysts) thought to occur due to abnormal development of the lymphatic system.

Lymphatic System

The lymphatic system consists of a fluid (lymph), vessels that transport the lymph, and organs that contain lymphoid tissue.

Lymph Nodes

A lymph node is a rounded mass of lymphatic tissue that is surrounded by a capsule of connective tissue. Lymph nodes filter lymph (lymphatic fluid), and they store lymphocytes (white blood cells). They are located along lymphatic vessels.

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Unicentric Castleman Disease

Unicentric Castleman disease (UCD) is a rare condition that affects the lymph nodes and related tissues. It is a form of Castleman disease that is "localized" to a single set of lymph nodes.

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