Castleman Disease – Symptoms and Causes

ByJanis Updated

Overview

Castleman disease refers to a group of uncommon disorders characterized by enlarged lymph nodes and various symptoms. The disease has different forms based on how many lymph nodes are affected and what symptoms appear.

Unicentric Castleman disease (UCD) affects just one lymph node, typically found in the chest or neck, though it can appear elsewhere in the body. This is the most common form of the condition.

Multicentric Castleman disease (MCD) is more complex, involving multiple enlarged lymph nodes, inflammatory symptoms, and potential organ function problems. MCD falls into three categories:

  • HHV-8-Associated MCD: Connected to human herpesvirus type 8 and HIV.
  • Idiopathic MCD: Has no known cause (also called HHV-8-negative MCD). iMCD-TAFRO is the most severe form of this type, named after its distinctive symptoms.
  • POEMS-Associated MCD: Linked to POEMS syndrome, a rare blood disorder that damages nerves.

A newly recognized subtype called oligocentric Castleman disease involves 2-3 enlarged lymph nodes with mild symptoms that don’t meet the criteria for MCD. This subtype remains rare and may be confused with other conditions.

The outlook for patients differs based on which form they have. Unicentric cases generally respond well to surgical removal of the affected lymph node.

For patients with MCD, while not everyone responds to initial treatment, effective medications exist for both the HHV-8-associated and idiopathic types.

Symptoms and Observations

Physical Changes

Castleman disease can cause various physical changes that differ from person to person. Swollen lymph nodes may appear with no other issues in some people.

These enlarged glands might be discovered during a regular checkup or when getting images for different health concerns.

Other physical symptoms can include fevers, losing weight without trying, feeling very tired, sweating at night, general swelling, and a larger liver or spleen.

When Medical Help Is Needed

You should contact your healthcare provider if you notice an enlarged lymph node in your neck, armpit, collarbone area, or groin.

You should also call if you feel ongoing fullness in your chest or belly, have unexplained fever, feel constantly tired, or lose weight without trying. These symptoms need proper medical evaluation to determine their cause.

Causes

The exact cause of unicentric Castleman disease remains unknown. Similarly, idiopathic multicentric Castleman disease (MCD) has no clear origin.

However, research shows that HHV-8-positive MCD typically develops in people with compromised immune systems, particularly those with HIV infection or other immune system disorders.

Risk Factors

Castleman disease can affect anyone, regardless of age or gender. Most diagnoses occur in middle age, though it can develop at any life stage, including childhood.

For unicentric Castleman disease and idiopathic multicentric Castleman disease, no specific risk factors have been identified.

However, for HHV-8-positive multicentric Castleman disease, two main risk factors exist:

  • HIV infection
  • Conditions that weaken the immune system

These factors increase a person’s chance of developing this specific type of Castleman disease.

Complications

UCD patients typically have good outcomes after the affected lymph node is removed, with normal life expectancy.

However, they face an increased risk of developing paraneoplastic pemphigus, a rare but serious autoimmune condition that causes blisters in the mouth and on the skin.

These symptoms are often misdiagnosed. The risk is low, but screening for paraneoplastic pemphigus is important for anyone with UCD.

Idiopathic multicentric Castleman disease can deteriorate rapidly, leading to serious organ function problems that may require:

  • Ventilator support for breathing
  • Dialysis for kidney function
  • Blood transfusions

HHV-8-positive multicentric Castleman disease presents different challenges, including:

  • Potentially life-threatening infections
  • Organ failure
  • Worse outcomes for patients who also have HIV/AIDS

Regular monitoring and prompt medical attention are essential for managing these complications effectively. Early intervention by healthcare providers can help improve outcomes for patients with all forms of Castleman disease.


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Health Writer

Janis's areas of expertise include nutrition, exercise, stress management, and mental health. She is passionate about educating and empowering people to take control of their health and make positive changes that can last a lifetime. Janis believes that small, sustainable changes are the key to long-term success, and she works with her clients to develop personalized plans that fit their individual needs and lifestyles.