Castleman Disease: Types, Symptoms, Treatment & Complete Guide
Updated: 2 days ago
Medically reviewed by Dr. Baraa Alnahhal, MD · Last reviewed: October 2026
Quick Answer: What Is Castleman Disease?
Castleman disease is a group of rare disorders involving enlarged lymph nodes and a wide range of symptoms. Unicentric Castleman disease affects a single lymph node and can usually be treated successfully with surgery, while multicentric Castleman disease affects multiple regions and causes inflammatory symptoms. Diagnosis requires a lymph node biopsy, and treatment depends on the type. [1]
What the research says
Complete surgery is the preferred first step for unicentric disease. International consensus guidelines from 42 experts in 10 countries conclude that complete surgical resection is often curative and is the preferred first-line therapy when possible; asymptomatic unresectable UCD may be observed, and siltuximab should be considered for unresectable UCD with an inflammatory syndrome. (van Rhee et al., Blood Adv, 2020) [3]
Siltuximab beat placebo in the only randomized trial of multicentric disease. Among 79 HIV-negative, HHV-8-negative patients with symptomatic MCD, durable tumor and symptom responses occurred in 18 of 53 patients (34%) on siltuximab versus 0 of 26 on placebo (difference 34.0%, 95% CI 11.1–54.8, p = 0.0012), with similar rates of serious adverse events. (van Rhee et al., Lancet Oncol, 2014) [4]
Guidelines put anti-interleukin-6 therapy first for idiopathic MCD. A 42-expert international group that reviewed treatment effectiveness in 344 cases recommended siltuximab (or tocilizumab if siltuximab is unavailable), with or without corticosteroids, as preferred first-line therapy, and added combination chemotherapy for the most severe cases. (van Rhee et al., Blood, 2018) [5]
Castleman Disease at a Glance
What it is: A group of rare disorders involving enlarged lymph nodes and a wide range of symptoms
Most common form: Unicentric Castleman disease (UCD) — one enlarged node, usually in the chest or neck
More extensive form: Multicentric Castleman disease (MCD) — multiple node regions, inflammatory symptoms, organ problems
Other forms: POEMS-associated MCD; the recently described oligocentric subtype (rare)
Who it affects: Any age or gender — usually diagnosed in midlife, but can occur at any age including childhood
Key symptoms: Fever, weight loss, fatigue, night sweats, swelling, anemia, abnormal blood proteins
Essential diagnostic test: Lymph node biopsy — also rules out lymphoma
Treatment: Depends on type — surgery for UCD; rituximab or siltuximab-based medicine for MCD
Outlook (UCD): Usually cured by surgery; life expectancy usually unchanged
Castleman disease at a glance: the four forms, symptoms, diagnosis, treatment by type, and complications.
What Is Castleman Disease?
Castleman disease is a group of rare disorders that involves lymph nodes that get bigger, called enlarged lymph nodes, and a wide range of symptoms.
The most common form involves a single enlarged lymph node. This lymph node is usually in the chest or neck, but it can occur in other areas of the body as well. This form is called unicentric Castleman disease (UCD).
Multicentric Castleman disease (MCD) involves multiple regions of enlarged lymph nodes, inflammatory symptoms, and problems with organ function. There are three types of MCD, each with its own driver:
HHV-8-associated MCD: Linked to human herpesvirus type 8 (HHV-8) and human immunodeficiency virus (HIV).
Idiopathic MCD: Cause is unknown — also called HHV-8-negative MCD; the most serious form is iMCD-TAFRO, named for the symptoms it causes.
POEMS-associated MCD: Linked to POEMS syndrome, a rare blood disorder that damages nerves and affects other parts of the body.
The four forms of Castleman disease: unicentric, multicentric (three subtypes), and the rare oligocentric subtype.
The Rare Fourth Form: Oligocentric Castleman Disease
Sometimes, people have 2–3 enlarged lymph nodes and mild symptoms that do not meet the diagnostic criteria for MCD. These people may have another disease entirely, or they may have the recently described subtype called oligocentric Castleman disease. This subtype is rare, and the best treatment for it is not known but is thought to be similar to the treatment for unicentric Castleman disease.
What are the symptoms of Castleman disease?
Castleman disease is often silent, especially the unicentric type, but when symptoms appear they fall into body symptoms such as fever and fatigue and blood symptoms such as anemia.
Unicentric Castleman Disease
Many people with UCD don't notice any signs or symptoms. The enlarged lymph node may be found during a physical exam or an imaging test for a different problem. Some people with UCD do develop symptoms more often seen in multicentric Castleman disease — listed below. [1]
Body Symptoms and Blood Symptoms
When symptoms appear, they fall into two groups:
Body symptoms: Fever; weight loss that happens without trying; fatigue; night sweats; swelling; enlarged liver or spleen.
Blood symptoms: Low red blood cell count (anemia); high or low platelet counts; higher creatinine levels because the kidneys don't work properly; higher levels of antibodies called immunoglobulins; low levels of the blood protein albumin.
The iMCD-TAFRO Symptom Set
Symptoms of the more serious form of idiopathic MCD, called iMCD-TAFRO, include:
Low platelet count: Also called thrombocytopenia — affects clotting.
Swelling and fluid in the body: Also called anasarca.
Fever or higher C-reactive protein: A marker of inflammation.
Reticulin fibrosis: Checked by taking a sample of bone marrow.
Organ swelling: Also called organomegaly.
When should you see a doctor about Castleman disease?
If you notice an enlarged lymph node on the side of your neck or in your underarm, collarbone, or groin area, talk with your healthcare professional. Also call your care team if you have a lasting feeling of fullness in your chest or abdomen, or fever, fatigue, or weight loss that you can't explain.
Because UCD often has no symptoms at all, this guidance matters: a persistent lump or a cluster of vague inflammatory symptoms is exactly the reason to get checked — and the workup can distinguish Castleman disease from other lymph node disorders.
What causes Castleman disease?
It's not clear what causes unicentric Castleman disease or idiopathic multicentric Castleman disease. However, HHV-8-positive MCD is known to occur in people whose immune systems do not have typical function because of HIV or other causes. [1]
What are the risk factors for Castleman disease?
Castleman disease can affect people of any age or gender. People usually are diagnosed during midlife, but it can happen at any age — including during childhood.
Unicentric Castleman disease (UCD): None known.
Idiopathic MCD: None known.
HHV-8-positive MCD: HIV infection, or a condition that decreases how well the immune system works, raises the risk.
What complications can Castleman disease cause?
Castleman disease complications differ by type, ranging from a low risk of paraneoplastic pemphigus in unicentric disease to rapidly worsening, life-threatening organ problems in idiopathic multicentric disease.
UCD: People usually do well once the affected lymph node is removed, and life expectancy is usually not changed. However, they are at increased risk of a rare autoimmune condition called paraneoplastic pemphigus — it can be life-threatening, causing blisters in the mouth and on the skin that are often misdiagnosed. The risk is low, but being checked for this condition is important if you have UCD. UCD also sometimes comes back after treatment.
Idiopathic MCD: Can rapidly worsen to life-threatening organ function problems, requiring critical care with a ventilator (a machine that helps with breathing) and treatments that help organs function, such as dialysis and transfusions.
HHV-8-positive MCD: May involve life-threatening infections and organ failure; people who also have HIV/AIDS generally have worse outcomes.
How Is Castleman Disease Diagnosed?
After reviewing your medical history and doing a detailed physical exam, your healthcare professional may recommend: [2]
Blood and urine tests: Help rule out other infections or diseases; can find anemia and changes in blood proteins that are typical of Castleman disease.
Imaging tests: Find enlarged lymph nodes or an enlarged liver or spleen. A CT scan of the neck, chest, abdomen, and pelvis may be used. A PET scan may be used to diagnose Castleman disease — and to show whether a treatment is working.
Lymph node biopsy: Essential to diagnose Castleman disease and rule out related disorders such as lymphoma — a tissue sample from an enlarged lymph node is removed and examined in a laboratory.
How is Castleman disease treated?
Treatment depends on the type of Castleman disease you have. [2]
Unicentric Castleman Disease
Surgery to remove the affected lymph node is the usual treatment for UCD. If the lymph node is in the chest or abdomen, major surgery may be needed.
Surgery usually cures UCD — however, UCD sometimes comes back. If surgery is not possible, you may need medicines typically used for multicentric Castleman disease. If medicines don't work, radiation therapy may be an option.
More research into oligocentric Castleman disease is needed, but treatment is usually similar to that of UCD.
You'll likely need follow-up exams, including imaging and laboratory tests, to check that the disease hasn't come back.
HHV-8-Positive Multicentric Castleman Disease
Rituximab (Rituxan) is usually the first treatment for HHV-8-positive MCD, and it is highly effective. Sometimes antivirals and chemotherapies are needed — antiviral medicines can block the activity of HHV-8 or HIV, and chemotherapies can get rid of extra immune cells.
Idiopathic Multicentric Castleman Disease
Siltuximab (Sylvant) is usually the first treatment for idiopathic MCD. In the U.S., it is the only medicine approved by the Food and Drug Administration (FDA) for treating idiopathic MCD. People who get better after taking siltuximab tend to have successful long-term treatment.
How it works: this drug blocks the action of a protein called interleukin-6 — and the bodies of people with idiopathic MCD produce too much of this protein.
People who are critically ill with idiopathic MCD often receive corticosteroids — such as prednisone — to help control inflammation, and they may also need chemotherapy to get rid of the immune cells that are causing problems.
When siltuximab doesn't work, other treatments such as rituximab (Rituxan) and sirolimus (Rapamune) may be used.
Treatment Summary
UCD: First-line — surgery to remove the affected node. If it isn't enough — MCD medicines; then radiation therapy.
HHV-8-positive MCD: First-line — rituximab (Rituxan), highly effective. If it isn't enough — antivirals and/or chemotherapy.
Idiopathic MCD: First-line — siltuximab (Sylvant), which blocks interleukin-6. If it isn't enough — corticosteroids (prednisone) and chemotherapy when critical; rituximab or sirolimus as backups.
How should you prepare for a Castleman disease appointment?
You may be referred to a doctor trained in treating blood disorders — this type of doctor is called a hematologist.
What You Can Do
Write down the symptoms you have been experiencing and for how long.
Write down key medical information, including other health conditions.
Make a list of all medicines, vitamins, and supplements you're taking, including the doses.
Questions to Ask
What's the most likely cause of my symptoms?
What kinds of tests do I need? Do they require any special preparation?
What treatment do you recommend? Do I need surgery?
In addition to the questions you've prepared, don't hesitate to ask other questions during your appointment.
What to Expect from Your Doctor
Your healthcare team is likely to ask you a number of questions, such as:
When did you first have symptoms?
Have your symptoms been continuous or occasional?
How bad are your symptoms?
Does anything seem to improve your symptoms?
What, if anything, appears to worsen your symptoms?
Do you have any other health conditions, such as lymphoma, HIV/AIDS, or Kaposi sarcoma?
Frequently Asked Questions
What is Castleman disease?
Castleman disease is a group of rare disorders that involves enlarged lymph nodes and a wide range of symptoms. The most common form (unicentric) involves a single enlarged node, usually in the chest or neck, while the multicentric form involves multiple regions of nodes, inflammatory symptoms, and organ function problems.
Is Castleman disease a type of cancer?
Castleman disease is described as a group of rare disorders — not as a cancer. But a lymph node biopsy is essential at diagnosis partly to rule out related disorders such as lymphoma, a blood cancer.
What causes Castleman disease?
It's not clear what causes unicentric Castleman disease or idiopathic multicentric Castleman disease. HHV-8-positive multicentric Castleman disease is known to occur in people with atypical immune system function because of HIV or other causes, and POEMS-associated MCD is linked to POEMS syndrome.
What are the symptoms of Castleman disease?
Many people with unicentric Castleman disease have no symptoms. When they appear, symptoms can include fever, unexplained weight loss, fatigue, night sweats, swelling, enlarged liver or spleen, anemia, abnormal platelet counts, higher creatinine, higher immunoglobulins, and low albumin.
How is Castleman disease treated?
Treatment depends on the type: surgery usually cures unicentric Castleman disease; rituximab is usually the first treatment for HHV-8-positive multicentric disease, sometimes with antivirals and chemotherapy; and siltuximab — the only FDA-approved medicine for idiopathic multicentric Castleman disease in the U.S. — is usually the first treatment for that type. [2]
Is surgery always needed?
Surgery to remove the affected lymph node is the usual treatment for unicentric Castleman disease, but it isn't always possible. If surgery can't be done, medicines used for multicentric disease may help, and radiation therapy is an option when medicines don't work.
Does Castleman disease come back after treatment?
Unicentric Castleman disease sometimes comes back after surgery, which is why follow-up exams — including imaging and laboratory tests — are important. People with multicentric forms need ongoing treatment tailored to their type.
Conclusion
The three lessons to carry away from this guide:
The type decides everything: one enlarged node (UCD) usually means surgery and a cure; multiple node regions (MCD) mean medicine-driven care that targets the specific driver — HHV-8, excess interleukin-6, or POEMS syndrome.
Silence is common, so symptoms matter: many people with UCD never notice anything, so an unexplained lump in the neck, underarm, collarbone, or groin — or unexplained fever, fatigue, and weight loss — is the moment to talk to a healthcare professional.
The biopsy is the gatekeeper: a lymph node biopsy is essential not just to confirm Castleman disease, but to rule out related disorders such as lymphoma — and each type then follows its own proven treatment path.
This guide has walked you through the four forms of Castleman disease, the body and blood symptoms to watch for, the special iMCD-TAFRO symptom set, who is at risk, the diagnosis process, the type-by-type treatment ladder, and the complications that make follow-up care essential.
This article is for informational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition.
References
Mayo Clinic Staff. Castleman disease — Symptoms and causes. Mayo Clinic. September 18, 2026. https://www.mayoclinic.org/diseases-conditions/castleman-disease/symptoms-causes/syc-20543017
Mayo Clinic Staff. Castleman disease — Diagnosis and treatment. Mayo Clinic. September 18, 2026. https://www.mayoclinic.org/diseases-conditions/castleman-disease/diagnosis-treatment/drc-20543055
van Rhee F, Oksenhendler E, Srkalovic G, et al. International evidence-based consensus diagnostic and treatment guidelines for unicentric Castleman disease. Blood Adv. 2020;4(23):6039-6050. PMID 33284946. https://doi.org/10.1182/bloodadvances.2020003334
van Rhee F, Wong RS, Munshi N, et al. Siltuximab for multicentric Castleman's disease: a randomised, double-blind, placebo-controlled trial. Lancet Oncol. 2014;15(9):966-974. PMID 25042199. https://doi.org/10.1016/S1470-2045(14)70319-5
van Rhee F, Voorhees P, Dispenzieri A, et al. International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease. Blood. 2018;132(20):2115-2124. PMID 30181172. https://doi.org/10.1182/blood-2018-07-862334
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