Multicentric Castleman s DiseaseNCT04968288
Natural History of KSHV-Associated Multicentric Castleman s Disease
An observational study of Multicentric Castleman s Disease, run by National Cancer Institute (NCI) at 1 site in United States.
Trial summary
Background: Kaposi s sarcoma herpes virus (KSHV) causes several kinds of cancer, Kaposi sarcoma (KS), a form of Multicentric Castleman s Disease (MCD) and a type of lymphoma known as Primary Effusion Lymphoma (PEL). These cancers can occur alone or at the same time in the same patient. Read more about this study
- Who is it for?
- Ages 18 years and older
- Conditions the study is for
- Multicentric Castleman s Disease
- How many people
- 195 participants
- Where
- 1 siteUnited States
- Study dates
- 17 Nov 2021to 1 Jan 2035
- Phase
- Observational
- Are there results?
- Not yet: the study is under way
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 18 years and older
- All sexes
- No healthy volunteers
- Adult, Older adult
Study population: primary clinical
Who may be able to join
- Biopsy proven KSHV-associated MCD, confirmed in the Laboratory of Pathology (LP), CCR
- Age >=18 years.
- ECOG performance status <=4 (Karnofsky >=20%).
- Ability of subject to understand and the willingness to sign a written informed consent document.
- Participants may be co-enrolled in other protocols, including investigational studies to treat KSHV-associated malignancies.
Who cannot join
- Any condition or set of circumstances that in the opinion of the investigators would make participation in this study unsafe or otherwise inappropriate for a given individual.
- Pregnancy
Trial locations and contacts
1 site in 1 country. Contact the study team or a site near you to ask about taking part.
United States1 site, 1 recruiting
- Recruiting
National Institutes of Health Clinical Center
Bethesda, Maryland, 20892
For more information at the NIH Clinical Center contact National Cancer Institute Referral Office, 888-624-1937
About this study
Official title: Natural History Study of KSHV-Associated Multicentric Castleman's Disease
Background:
Kaposi s sarcoma herpes virus (KSHV) causes several kinds of cancer, Kaposi sarcoma (KS), a form of Multicentric Castleman s Disease (MCD) and a type of lymphoma known as Primary Effusion Lymphoma (PEL). These cancers can occur alone or at the same time in the same patient. MCD can cause a lot of symptoms and problems with various organs in the body, making patients feel quite unwell. If unrecognized, the disease can be fatal. Medications such as rituximab alone or in combination with chemotherapy may help treat MCD but there is little known about the long term effects and the natural course of MCD.
Read more
Objective:
To better understand the biology of KSHV-MCD to help identify how this disease causes illness and how cancer treatments known to be effective in MCD may help patients with this condition. This study also aims to help identify ways to treat the disease by providing other standard cancer treatments that would be useful to use to treat MCD based on what we know about this condition.
Eligibility:
People 18 years of age and older with KSHV-MCD.
Design:
Participants will be screened with:
Medical history
Physical exam
CT scan
Blood and heart tests
Participants will have an initial evaluation. This will include:
Review of participants symptoms and ability to perform their normal activities
Blood and urine tests
Imaging studies such as CT and PET scans. Participants may have a contrast agent injected into their arm.
Photographs to document skin lesions
Optional skin biopsy. For this, a small piece of the skin will be removed.
Optional lymph node needle biopsy
Optional samples of the fluid in the space around the lungs, intestines, or heart
Optional sample of the liquid that surrounds the brain and spinal cord
Saliva samples
DEXA scan to examine the bones
Questionnaires
Optional limb measurements or cognitive tests
Physicians will give participants recommendations about treatment.
After their initial evaluation and any treatment, participants will have additional visits. These will occur every 3 months for the first year, then every 6 months for the second year, and then once a year for up to 1 year.
Read the detailed description
Background:
- Multicentric Castleman s disease (MCD) is a rare but lethal Kaposi s sarcoma-associated herpesvirus (KSHV) associated lymphoproliferative disorder with a historical median survival of 2 years. It occurs more often in HIV-infected individuals than those without HIV infection. The poor prognosis is not fully explained by the underlying HIV, as the HIV-negative cases appear to have little or no survival advantage over the HIV-positive cohort. The disease has no defined standard treatment and there is much to be learned about its pathogenesis and natural history with current treatments.
- Patients with KSHV-MCD may present with other KSHV-associated conditions such as Kaposi sarcoma (KS) and may develop primary effusion lymphoma (PEL) following their diagnosis, which may affect morbidity and mortality. It is not clear how these concurrent conditions develop simultaneously and over time.
- Since 2004, the HIV and AIDS Malignancy Branch (HAMB) has studied the natural history and assessed novel therapies for KSHV-MCD which has contributed to the development of at least two therapies that are now considered as first-line therapies: (1) rituximab plus liposomal doxorubicin and (2) high dose zidovudine plus valganciclovir.
- These therapies plus either rituximab alone or other rituximab-based therapies that have been developed over the last 15 years have resulted in durable responses in patients with KSHV-MCD and have substantially improved the prognosis of these patients.
- The longitudinal evaluations along with tissue sample collection, imaging studies, and participant reported outcomes will allow for the development of a better understanding of the natural history of this disease, and the development of the basis for more effective treatments.
Objective:
-To study and describe the natural history of KSHV-MCD
Eligibility:
- Age >= 18 years
- Biopsy proven KSHV-associated MCD
Design:
- This will be a long-term study to comprehensively study participants with KSHV-MCD
- Medical history will be collected, and participants followed throughout the course of their illness, with particular attention to patterns of disease presentation, recurrence and progression, response to therapies, and duration of responses
- Tissue samples and blood will be obtained from participants during this study.
- We anticipate accruing 135 evaluable participants on this protocol
- Conditions
- Multicentric Castleman s Disease
- Keywords
- Kaposi s sarcoma, HIV, primary effusion lymphoma, interlerleukin-6, Natural History
- Medical subject headings
- Lymphoma, Primary Effusion
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
| Study group | Role | What they receive |
|---|---|---|
| Cohort 1 | Subjects with KSHV-associated MCD |
What is being measured
The primary outcome is the main question the study is designed to answer.
natural history of MCD
clinical presentation, patterns of disease progression, therapeutic response, disease recurrence and participant overall survival
Measured: ongoing
Secondary outcomes (3)
concurrent KS or PEL evaluation
Percentage of subjects with concurrent PEL or KS
Measured: every 3 months for year 1, every 6 month for year 2, yearly from year 3
relapse-free survival
time from any treatment until first recurrence
Measured: every 3 months for year 1, every 6 month for year 2, yearly from year 3
overall survival
time from any treatment to death
Measured: every 3 months for year 1, every 6 month for year 2, yearly from year 3
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted20 Jul 2021
- Study start17 Nov 2021
- Primary completion1 Jan 2034 (estimated)
- Study completion1 Jan 2035 (estimated)
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Other trials for Multicentric Castleman s DiseaseStudies of the same condition, open and under way.
- More trials run by National Cancer Institute (NCI)The sponsor's other registered studies.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Observational
- Observational model
- Cohort
- Time perspective
- Prospective
- Enrolment
- 195 participants (estimated)
Sponsor and investigators
- Lead sponsor
- National Cancer Institute (NCI)
- Sponsor type
- US National Institutes of Health
- Responsible party
- Sponsor
- Investigators
- Ramya M Ramaswami, M.D., Principal investigator, National Cancer Institute (NCI)
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT04968288
- Sponsor's protocol ID
- 10000337
- Other IDs
- 000337-C
- Status last verified
- 13 Oct 2026
- First submitted
- 17 Jul 2021
- Last updated
- 16 Oct 2026
- Results posted
- No
- FDA-regulated drug
- No
- FDA-regulated device
- No
Study at a glance
From the registry record, last updated by the sponsor on 16 Oct 2026. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Natural History Study of KSHV-Associated Multicentric Castleman's Disease
- ClinicalTrials.gov ID
- NCT04968288
- Acronym / sponsor ID
- 10000337
- Conditions
- Multicentric Castleman s Disease
- Keywords
- Kaposi s sarcoma, HIV, primary effusion lymphoma, interlerleukin-6, Natural History
- Study type
- Observational
- Recruitment status
- Recruiting
- Enrolment
- 195 participants (estimated)
- Lead sponsor
- National Cancer Institute (NCI)US National Institutes of Health
- Study start
- 17 Nov 2021 (actual)
- Primary completion
- 1 Jan 2034 (estimated)
- Study completion
- 1 Jan 2035 (estimated)
- First posted
- 20 Jul 2021 (actual)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
Background:
Kaposi s sarcoma herpes virus (KSHV) causes several kinds of cancer, Kaposi sarcoma (KS), a form of Multicentric Castleman s Disease (MCD) and a type of lymphoma known as Primary Effusion Lymphoma (PEL). These cancers can occur alone or at the same time in the same patient. MCD can cause a lot of symptoms and problems with various organs in the body, making patients feel quite unwell. If unrecognized, the disease can be fatal. Medications such as rituximab alone or in combination with chemotherapy may help treat MCD but there is little known about the long term effects and the natural course of MCD.
Objective:
To better understand the biology of KSHV-MCD to help identify how this disease causes illness and how cancer treatments known to be effective in MCD may help patients with this condition. This study also aims to help identify ways to treat the disease by providing other standard cancer treatments that would be useful to use to treat MCD based on what we know about this condition.
Eligibility:
People 18 years of age and older with KSHV-MCD.
Design:
Participants will be screened with:
Medical history
Physical exam
CT scan
Blood and heart tests
Participants will have an initial evaluation. This will include:
Review of participants symptoms and ability to perform their normal activities
Blood and urine tests
Imaging studies such as CT and PET scans. Participants may have a contrast agent injected into their arm.
Photographs to document skin lesions
Optional skin biopsy. For this, a small piece of the skin will be removed.
Optional lymph node needle biopsy
Optional samples of the fluid in the space around the lungs, intestines, or heart
Optional sample of the liquid that surrounds the brain and spinal cord
Saliva samples
DEXA scan to examine the bones
Questionnaires
Optional limb measurements or cognitive tests
Physicians will give participants recommendations about treatment.
After their initial evaluation and any treatment, participants will have additional visits. These will occur every 3 months for the first year, then every 6 months for the second year, and then once a year for up to 1 year.
Detailed description
Background:
- Multicentric Castleman s disease (MCD) is a rare but lethal Kaposi s sarcoma-associated herpesvirus (KSHV) associated lymphoproliferative disorder with a historical median survival of 2 years. It occurs more often in HIV-infected individuals than those without HIV infection. The poor prognosis is not fully explained by the underlying HIV, as the HIV-negative cases appear to have little or no survival advantage over the HIV-positive cohort. The disease has no defined standard treatment and there is much to be learned about its pathogenesis and natural history with current treatments.
- Patients with KSHV-MCD may present with other KSHV-associated conditions such as Kaposi sarcoma (KS) and may develop primary effusion lymphoma (PEL) following their diagnosis, which may affect morbidity and mortality. It is not clear how these concurrent conditions develop simultaneously and over time.
- Since 2004, the HIV and AIDS Malignancy Branch (HAMB) has studied the natural history and assessed novel therapies for KSHV-MCD which has contributed to the development of at least two therapies that are now considered as first-line therapies: (1) rituximab plus liposomal doxorubicin and (2) high dose zidovudine plus valganciclovir.
- These therapies plus either rituximab alone or other rituximab-based therapies that have been developed over the last 15 years have resulted in durable responses in patients with KSHV-MCD and have substantially improved the prognosis of these patients.
- The longitudinal evaluations along with tissue sample collection, imaging studies, and participant reported outcomes will allow for the development of a better understanding of the natural history of this disease, and the development of the basis for more effective treatments.
Objective:
-To study and describe the natural history of KSHV-MCD
Eligibility:
- Age >= 18 years
- Biopsy proven KSHV-associated MCD
Design:
- This will be a long-term study to comprehensively study participants with KSHV-MCD
- Medical history will be collected, and participants followed throughout the course of their illness, with particular attention to patterns of disease presentation, recurrence and progression, response to therapies, and duration of responses
- Tissue samples and blood will be obtained from participants during this study.
- We anticipate accruing 135 evaluable participants on this protocol
Study design
- Observational model
- Cohort
- Time perspective
- Prospective
- Number of arms
- 1
| Arm | Type | Receives |
|---|---|---|
| Cohort 1 | – | –Subjects with KSHV-associated MCD |
Who may qualify
- Ages
- 18 years and older
- Sex
- All
- Healthy volunteers
- Not accepted
- Age groups
- Adult, Older adult
- Study population
- primary clinical
Key inclusion criteria (5 listed)
- Biopsy proven KSHV-associated MCD, confirmed in the Laboratory of Pathology (LP), CCR
- Age >=18 years.
- ECOG performance status <=4 (Karnofsky >=20%).
- Ability of subject to understand and the willingness to sign a written informed consent document.
- Participants may be co-enrolled in other protocols, including investigational studies to treat KSHV-associated malignancies.
Key exclusion criteria (2 listed)
- Any condition or set of circumstances that in the opinion of the investigators would make participation in this study unsafe or otherwise inappropriate for a given individual.
- Pregnancy
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Primary outcome
natural history of MCD
Time frame: ongoing
clinical presentation, patterns of disease progression, therapeutic response, disease recurrence and participant overall survival
3 secondary outcomes
concurrent KS or PEL evaluation
Time frame: every 3 months for year 1, every 6 month for year 2, yearly from year 3
relapse-free survival
Time frame: every 3 months for year 1, every 6 month for year 2, yearly from year 3
overall survival
Time frame: every 3 months for year 1, every 6 month for year 2, yearly from year 3
Results and safety
This study is still under way. Results are not available in the source record yet.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 16 Oct 2026.
- NIH Clinical Center Detailed Web PageLink provided by the sponsor.
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Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part.
Ramya M Ramaswami, M.D. principal investigator
National Cancer Institute (NCI)
1 site in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
An observational study of Multicentric Castleman s Disease, run by National Cancer Institute (NCI) at 1 site in United States.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 5 inclusion and 2 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: looking for participants now, as of the sponsor's last update on 16 Oct 2026. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 1 site in United States. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
This study is still under way. Results are not available in the source record yet.
How can a doctor refer a patient or contact the research team?
Contact Irene B Ekwede, R.N. ((240) 760-6126, irene.ekwede@nih.gov), or a site near the patient. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
natural history of MCD (time frame: ongoing). It is what the study was designed to measure, not a finding.
Where can I read the original study information?
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 16 Oct 2026; AzTrials last refreshed its copy on 26 Oct 2026.