Allogeneic bone marrow transplantation for active Epstein-Barr virus-related lymphoproliferative disease and hemophagocytic lymphohistiocytosis in an infant with severe combined immunodeficiency syndrome.
Bone Marrow Transplant
; 29(6): 519-21, 2002 Mar.
Article
em En
| MEDLINE
| ID: mdl-11960273
ABSTRACT
A 5-month-old male presented with fever, hepatosplenomegaly, leukocytosis with atypical lymphoblasts, anemia and thrombocytopenia. Severe combined imunodeficiency syndrome (T-, B+, NK+), B lymphoproliferative disease and hemophagocytic lymphohistiocytosis triggered by Epstein-Barr virus (EBV) were diagnosed. As his clinical situation deteriorated rapidly, BMT was performed with unmanipulated marrow stem cells from his EBV-positive HLA-identical sister after conditioning with dexamethasone (1.75 mg/kg/day), cyclophosphamide (114 mg/kg) and etoposide (10 mg/kg), with no immunosuppression given post transplant. Engraftment occurred on day 6 with explosive proliferation of donor CD8(+) T cells. The patient died 3 days later from acute respiratory distress syndrome. Autopsy revealed full donor engraftment and no signs of hemophagocytic lymphohistiocytosis or B lymphoproliferative disease. Thus, transplanted T cells can expand very rapidly within days after BMT and clear EBV lymphoproliferative disease and hemophagocytic lymphohistiocytosis.
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Base de dados:
MEDLINE
Assunto principal:
Histiocitose de Células não Langerhans
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Transplante de Medula Óssea
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Imunodeficiência Combinada Severa
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Herpesvirus Humano 4
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Infecções por Vírus Epstein-Barr
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Transtornos Linfoproliferativos
Idioma:
En
Ano de publicação:
2002
Tipo de documento:
Article