Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:2.1.1.69 (BMT)
 2,655 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We describe the first reported Klinefelter's syndrome (KS) in which allogeneic bone marrow transplantation from an unrelated donor (UR-BMT) was performed for treatment of chronic myelogenous leukemia in blast crisis (CML-BC). A 31-year-old male patient was diagnosed as having CML-BC with KS in April 2001. The result of a bone marrow chromosomal examination were 47, XXY, t(9;22)(q34;q11). After he had been treated with chemotherapy and imatinib mesylate, he underwent UR-BMT in February 2002. After the UR-BMT, his bone marrow chromosome changed from 47, XXY, t(9;22)(q34;q11) to 46,XY and 100% donor-type chimerism was obtained. However, he relapsed on day 83 after UR-BMT. After treatment with imatinib mesylate and tapering of immunosuppressive agents, skin and liver GVHD developed and then donor-type chimerism was increased with decreased blast cells. However, the patient died due to progression of disease in October 2002.
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PMID:Allogeneic bone marrow transplantation from an unrelated donor for the treatment of chronic myelogenous leukemia in blast crisis in a patient with Kleinfelter's syndrome. 1516 Sep 66

WAS is a rare X-linked recessive disorder characterized by primary progressive T cell immunodeficiency, impaired antipolysaccharide antibody response, thrombocytopenia with small platelet, and eczematoid dermatitis. Untreated patients with typical WAS have poor prognosis with the major causes of death being infection, bleeding, lymphoproliferative disorders, and malignancy. Due to the increased risk of infectious and hemorrhagic episodes the best results with HSCT are achieved in patients less than five yr of age and are recommended as early as possible. Here, we report a three-yr-old boy with WAS who underwent UCB and BMT from his genotypically identical brother with Klinefelter syndrome.
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PMID:Hematopoietic stem cell transplantation from a donor with Klinefelter syndrome for Wiskott-Aldrich syndrome. 1853