ClinicalTrials.gov record NCT04968288
Natural History of KSHV-Associated Multicentric Castleman s Disease
Natural History Study of KSHV-Associated Multicentric Castleman's Disease
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AI-written from the registry record, not the study's own wording. May contain errors or omissions. Check the registry text on this page and the original record ↗ before acting on it.
This is a prospective observational study of adults with KSHV-associated Multicentric Castleman disease (MCD) to document how the disease behaves over time. Participants have an initial evaluation (medical history, physical exam, imaging, blood tests, and optional tissue or fluid samples) and are followed with visits every 3 months in year 1, every 6 months in year 2, and yearly thereafter. The study records clinical course, imaging, and samples to track presentation, recurrence, response to treatments, and survival. Physicians will provide treatment recommendations as appropriate.
What it is studying. The study aims to describe the natural history of KSHV-associated MCD: patterns of clinical presentation, disease progression and recurrence, response to therapies, occurrence of concurrent Kaposi sarcoma or primary effusion lymphoma, and overall survival.
What the researchers hope to learn. To define the clinical course of KSHV-associated MCD, document patterns of recurrence and response to therapies, measure concurrent KS or PEL occurrence and survival, and collect samples that can inform future research and treatment development.
What this study is about
The study's own summary, as published on ClinicalTrials.gov. Underlined terms have a short explanation.
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.
Eligibility
The criteria exactly as listed on ClinicalTrials.gov. Only the study team can say whether they apply to you.
Inclusion criteria
- 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.
Exclusion criteria
- 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
Study description and what participation involves
The study's detailed description, as published on ClinicalTrials.gov.
Show the detailed description from the registry
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
AI summaryWhat participation may involve, in plain languageShow ▾Hide ▴
AI-written from the registry record, not the study's own wording. May contain errors or omissions. Check the registry text on this page and the original record ↗ before acting on it.
Participants will have an initial evaluation including medical history, physical exam, review of symptoms and daily activity, blood and urine tests, imaging (CT and possibly PET scans with contrast), and heart tests. The study will collect blood and tissue samples and other specimens such as saliva; optional procedures may include a small skin biopsy, a needle biopsy of a lymph node, sampling of fluid from around the lungs/intestines/heart, and a spinal fluid sample (lumbar puncture). Photos of skin lesions, a DEXA bone scan, questionnaires, and optional limb measurements or cognitive tests may be done. After baseline, visits are scheduled every 3 months in year 1, every 6 months in year 2, and yearly thereafter.
How the study is structured. A prospective observational cohort: no experimental treatment is assigned. Participants receive baseline exams, imaging, and collection of blood and optional tissue or fluid samples, then regular follow-up visits (every 3 months in year 1, every 6 months in year 2, and yearly from year 3) with ongoing data and sample collection.
Questions you could ask
These questions are suggestions for a conversation with the study team or your own clinician, not advice.
- The registry lists this study under Multicentric Castleman s Disease. Does it apply to my diagnosis?
- This is an observational study, so the study itself gives no treatment. What would taking part involve for me, and would it change my usual care?
- The listed sites are in United States. Is any of them reachable for me, and does the study help with travel?
- Which of the eligibility criteria would need to be checked with tests before I could join?
More on Castleman disease
Important dates
- Study start
- Nov 17, 2021
- Primary completion
- Jan 1, 2034 (estimated)
- Study completion
- Jan 1, 2035 (estimated)
- Target enrollment
- 195 participants (estimated)
- Last updated on registry
- Aug 24, 2026
- Data verified
- Oct 10, 2026
Sponsor and research team
The lead sponsor funds and oversees the trial. A pharmaceutical or biotech company sponsor typically means the study is testing that company's drug or device; a hospital, university, or government agency usually indicates investigator-initiated or publicly funded research.
- Lead sponsor
- National Cancer Institute (NCI)
Central study contact
If you're interested in participating, the central contact can answer general questions about the study — including eligibility requirements and how to find a recruiting site near you. Actual eligibility is determined by the site's medical team.
- Irene B Ekwede, R.N. 📞 (240) 760-6126 ✉ irene.ekwede@nih.gov
- Ramya M Ramaswami, M.D. 📞 (240) 506-1088 ✉ ramya.ramaswami@nih.gov
Participating sites (1)
- National Institutes of Health Clinical CenterRecruiting
Bethesda, Maryland, 20892 · United States
- For more information at the NIH Clinical Center contact National Cancer Institute Referral Office · 888-624-1937
Terms used on this page · explanations by MedicalUpdates.ai
- CT scan
- An X-ray scan that builds detailed cross-section images of the body. 'High-resolution' CT (HRCT) is used to look closely at the lungs.
- PET
- A scan in which a small amount of a radioactive tracer is injected so that activity in tissues can be seen. Often combined with a CT or MRI scan.
- biopsy
- Removal of a small piece of tissue so it can be examined under a microscope. 'Biopsy-proven' or 'histologically confirmed' means the diagnosis was confirmed by examining tissue under a microscope.
- ECOG performance status
- A scale from 0 to 5 that describes how a person's condition affects daily activity. 0 means fully active; 1 means restricted in strenuous activity; 2 means able to self-care but unable to work; higher numbers mean more limitation.
- Karnofsky
- A scale from 100 (no complaints) down to 0 that describes how well a person can carry out daily activities. Studies often require a minimum score.
- written informed consent
- The process of explaining a study, including its risks and what participation involves, so that a person can decide freely whether to take part. Consent is usually documented in writing, and a participant can choose to leave the study later.
- investigational
- A treatment that is being tested and has not been approved for this use. It may be approved for a different condition. Being studied does not mean it works or is available outside the study.
- INCLUSION CRITERIA
- The conditions a person must meet to be considered for the study, for example a confirmed diagnosis, an age range or a test result.
- EXCLUSION CRITERIA
- The conditions that rule a person out even if they meet the inclusion criteria, for example other illnesses, certain medicines or pregnancy.
- cohort
- A group of people who share a characteristic, such as a diagnosis, and are studied together. 'Prospective' means they are followed forward from enrolment; 'retrospective' means existing records are reviewed.
- standard treatment
- Treatment generally considered appropriate for a condition in a particular clinical setting. It can vary by patient, place and over time. In many studies the new treatment is added to, or compared with, standard of care.
- natural history
- A study that follows how a condition changes over time without assigning an experimental treatment. It may involve study visits and tests, and is often used to plan future trials.
The study record on this page is reproduced from ClinicalTrials.gov, a database of the U.S. National Library of Medicine, where it was submitted by the study sponsor. MedicalUpdates.ai does not own this content, is not affiliated with or endorsed by ClinicalTrials.gov, NIH, or the U.S. Government, and does not verify the record. Plain-language notes, the glossary and the questions above are added by this site; text in the boxes marked "AI summary" was written by an AI model and may contain errors. Registry records change over time. Check the original record for the current version and discuss eligibility or participation with a qualified healthcare professional.
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