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ST. JUDE RESEARCHERS REPORT FIRST SUCCESSFUL TREATMENT OF DUNCAN'S SYNDROME

 /ADVANCE/MEMPHIS, Sept. 2 /PRNewswire/ -- The first successful treatment of a patient with Duncan's syndrome, a rare and fatal immune system disorder, has been reported by researchers at St. Jude Children's Research Hospital. This week's issue of the British scientific journal Lancet reports a study in which an 11-year-old boy was treated and seemingly cured using an allogeneic bone marrow transplant (one from a genetically matched donor who may or may not be related to the patient).
 Patients with X-linked lymphoproliferative disorder, known as "Duncan's syndrome," have a lethal immune system disorder that prevents them from fighting Epstein-Barr virus (EBV), a common virus that afflicts about 90 percent of the U.S. population at some time in their lives. In healthy people, the virus produces minor cold-like symptoms, such as swollen glands and sore throat, and is controlled by the immune system. In patients with Duncan's syndrome, the immune system cannot control the EBV and serious conditions, such as fevers and lymphoma (cancer), develop.
 "We're encouraged about the findings because we now know that bone marrow transplants can be safely performed on these patients," explained principal investigator Dr. Helen Heslop, department of hematology/oncology at St. Jude Hospital. "Although follow up will be required to determine if the patient is cured, the immune defects prior to transplant have been corrected."
 The patient in the study began chemotherapy for abdominal EBV-positive non-Hodgkin lymphoma at age 4. Between the ages of 4 and 11, he experienced two remissions and one relapse. His family history (an uncle died of the disease in his early teens) and other factors indicated he was at risk for developing further lymphomas, so at age 11 he received the bone marrow transplant. Presently, the patient, who is now 13 years old, is two years past the bone marrow transplant and shows no signs of the lymphoma and his bone marrow is normal.
 The donor for the transplant was his younger sister, whose bone marrow was genetically similar. The likelihood of having a matched sibling is one in four.
 The syndrome was identified in the early 1960s and was named after the first patient in whom it was identified. Since then, only a few hundred cases have been reported nationwide. Until now, most patients died before the age of 20.
 The cause of Duncan's syndrome is still unknown. "This study indicates that because the disorder was cured with an allogeneic bone marrow transplant, the primary abnormality lies somewhere in the bone marrow-derived cells of the immune system," Dr. Heslop said. Identifying the optimal time for a bone marrow transplant will be studied next. Gene therapy may also be a consideration.
 St. Jude Children's Research Hospital, founded by the late entertainer Danny Thomas, is an internationally recognized biomedical research center dedicated to finding cures for the catastrophic diseases of children. Its work is primarily supported through public funds raised by the American Lebanese Syrian Associated Charities (ALSAC). All St. Jude patients are treated regardless of their ability to pay, with ALSAC covering all costs beyond those reimbursed by third party insurers, and total costs when no insurance is available.
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 /NOTE TO EDITORS: The children of St. Jude appreciate your including a mention of Danny Thomas as founder in your story./
 /CONTACT: Jerry Chipman of St. Jude, 901-522-0306, or Sharon Monson of Fleishman-Hillard, 312-751-8878/


CO: St. Jude Children's Research Hospital ST: Tennessee IN: HEA SU:

SM -- NY053 -- 8401 09/02/93 12:03 EDT
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Date:Sep 2, 1993
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