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Post-transplant Lymphoproliferative Disease

Non-Langerhans-Cell Histiocytosis

Primarily transplant recipients undergoing monitoring for EBV and patients suspected for having PTLD.

Patients diagnosed with PTLD and other kinds of lymphoma. Patients undergoing hematopoietic stem cell transplantation and patients with hemophagocytosis.

Children undergoing transplantation. Children diagnosed with hemophagocytic lymphohistiocytosis.

Lene Ugilt, MD

Epstein-Barr virus (EBV) is one of several herpesviruses that cause disease in humans. EBV virus has an oncogenic potential, and it has been associated with the development of a wide range of cancers. Previous studies have shown a close association between EBV and Post-Transplant Lymphoproliferative disorder (PTLD) in transplant recipients. As part of a preventive approach against PTLD, several transplantation units now monitor the occurrence of EBV-DNAemia after transplantation. However, there is little evidence to guide this strategy; nor is there consensus concerning either the best specimen to use for EBV analysis (whole blood or plasma).

In this study investigators aim to optimise and validate a polymerase chain reaction (PCR)-test for EBV-DNA on, respectively, whole blood, plasma and a combination of plasma and lymphocytes.

The investigators wish to determine which of the three tests best predicts current and future risk of development of EBV-related diseases such as mononucleosis and PTLD.

EBV is one of several herpesviruses that cause disease in humans. Primary EBV infection usually occurs in early childhood and is generally asymptomatic, while later infection may cause mononucleosis. As with other herpesviruses, primary infection is followed by persistent (lifelong) infection. EBV virus has an oncogenic potential, and it has been associated with the development of a wide range of cancers. Previous studies have shown a close association between EBV and PTLD in transplant recipients. As part of a preventive approach against PTLD, several transplantation units now monitor the occurrence of EBV-DNAemia after transplantation. However, there is little evidence to guide this strategy; nor is there consensus concerning either the best specimen to use for EBV analysis (whole blood or plasma) or the appropriate clinical action to take if EBV-DNAemia is detected.

In this study investigators aim to optimise and validate a polymerase chain reaction (PCR)-test for EBV-DNA on, respectively, whole blood and a combination of plasma and lymphocytes.

Results obtained with the two new methods will be compared with those from the already established World Health Organization (WHO) standardised EBV-PCR test on ethylenediaminetetraacetic acid (EDTA)-plasma. The result of all three tests will be evaluated relative to EBV-related symptoms and other diseases.

The investigators wish to determine which of the three tests best predicts current and future risk of development of EBV-related diseases such as mononucleosis and PTLD.

Early Detection of Epstein-Barr Virus Related Disease.

Early Detection of Epstein-Barr Virus Related Disease. Improving Quantification of EBV in Blood Samples Using Polymerase Chain Reaction.

Symptoms compatible with EBV disease combined with a tissue biopsy positive for EBV

Symptoms compatible with EBV disease combined with a positive EBV-PCR

Aarhus University Hospital

University of Aarhus

Skin and Connective Tissue Diseases

Herpes Simplex

Chronic Graft Versus Host Disease

Chronic Active Epstein-Barr Virus Infection

Patients with chronic active Epstein-Barr virus.

Jeffrey I Cohen, M.D.

Epstein-Barr virus (EBV) is a member of the human herpes virus family that infects more than 95 percent of the U.S. population. Most infections occur in childhood and cause no symptoms; in adolescents and adults, EBV often causes infectious mononucleosis. It has also been associated with certain forms of cancer. Chronic Epstein-Barr virus (CAEBV) is a rare disease, primarily of children and young adults, that leads to life-threatening infections.

This study seeks to identify genetic mutations responsible for CAEBV. A secondary goal is to learn more about the natural history of CAEBV.

The study will examine blood and tissue samples from up to 50 patients (age 3 and above) with CAEBV and up to 150 of their relatives (age 1 and above). Autopsy samples may be included in the study. Up to 300 anonymous blood samples from the NIH Clinical Center Blood Transfusion Medicine will also be examined for comparison.

No more than 450 milligrams (30 tablespoons) of blood per 8 weeks will be drawn from adult patients, and no more than 7 milliliters per kilogram of blood per 8 weeks will be drawn from patients under age 18. Local health care providers will refer patients to the study and will obtain the samples. Some patients may also be seen at the NIH Clinical Center. Those patients will have a full medical history and physical examination, along with chest X-ray, blood counts, blood chemistry, EBV serologies, and viral load. Other tests, such as CT scan or MRI, may be performed if medically indicated. Patients will be asked to undergo leukapheresis.

In vitro tests on the blood or tissue samples will include analysis for proteins or genes that are involved in the immune response; cloning of portions of patient DNA; transformation of B cells with EBV; measurement of the ability of patient blood cells to kill EBV-infected cells; determination of lymphocyte subsets; and determination of antibodies to EBV or other herpes viruses.

If a genetic cause for CAEBV is found, the investigators will be available to discuss the results with patients in person or by telephone. Genetic indications of risk for other diseases will also be discussed with patients.

Patients with chronic active Epstein-Barr virus (CAEBV) have (a) an illness that began as a primary infection with EBV or markedly elevated titers of antibodies to EBV, (b) histological evidence of organ disease, and (c) elevated EBV DNA levels in the blood or EBV DNA or proteins in affected tissues. This is primarily a disease of children and young adults. Many of these patients develop hypogammaglobulinemia or pancytopenia and death is frequently due to opportunistic infections or B or T cell lymphoproliferative disease. The primary goal of this study is to identify genetic mutations responsible for CAEBV. The secondary goal of this study is to learn more about the natural history of CAEBV. Blood samples or available tissues (e.g. previous biopsy or autopsy material) from patients with CAEBV and their relatives will be analyzed to identify disease genes that are mutated in this population. In addition, some of the patients will be invited to come for evaluation at the NIH Clinical Center. At that time medically indicated studies of disease progression will be performed. Serial virologic and immunologic studies will be performed. Eligible relatives of patients will be studied to compare the gene(s) associated with CAEBV in patients with the gene(s) in their relatives. Knowledge gained from this study has the potential of providing insights into the immunologic control of EBV infections. In addition, identification of the molecular mechanisms for CAEBV may provide insights leading to improved treatments for this disease.

Study title	NCT ID	Conditions	Eligibility	Organization
Early Detection of Epstein-Barr Virus Related Disease.	NCT03546101	Post-transplant... Mononucleosis Epstein-Barr Vi... Epstein-Barr Vi... Epstein-Barr Vi... Epstein-Barr Vi... Epstein-Barr Vi... Hemophagocytic ... Hemophagocytosi...	-	University of Aarhus
Early Detection of Epstein-Barr Virus Related Disease.	NCT03546101	Post-transplant... Mononucleosis Epstein-Barr Vi... Epstein-Barr Vi... Epstein-Barr Vi... Epstein-Barr Vi... Epstein-Barr Vi... Hemophagocytic ... Hemophagocytosi...	-	University of Aarhus
Genetic Studies of Chronic Active Epstein-Barr Disease	NCT00032513	Chronic Active ...	1 Year - 120 Years	National Institutes of Health Clinical Center (CC)
Genetic Studies of Chronic Active Epstein-Barr Disease	NCT00032513	Chronic Active ...	1 Year - 120 Years	National Institutes of Health Clinical Center (CC)
Early Detection of Epstein-Barr Virus Related Disease.	NCT03546101	Post-transplant... Mononucleosis Epstein-Barr Vi... Epstein-Barr Vi... Epstein-Barr Vi... Epstein-Barr Vi... Epstein-Barr Vi... Hemophagocytic ... Hemophagocytosi...	-	University of Aarhus

Spots Global Cancer Trial Database for hemophagocytosis