RESUMO
A child with acute pre-B cell lymphoblastic leukemia underwent haploidentical bone marrow transplantation (BMT) after first relapse. Approximately 8 months after the BMT, he developed a soft tissue mass overlying a defect in the left frontal bone. He was found to have several additional osteolytic lesions but no evidence of lymphadenopathy or organomegaly. A biopsy of the presenting lesion demonstrated a polymorphous infiltrate composed predominantly of S-100 protein and CD68 immunoreactive histiocytic cells. Together with the presence of emperipolesis, the process was interpreted as Rosai-Dorfman (R-D) disease. He received chemotherapy with vinblastine, prednisone, 6-mercaptopurine and methotrexate and has been in remission for over 4 years. Only one previous example of acute lymphoblastic leukemia in childhood has been reported with R-D disease.
Assuntos
Transplante de Medula Óssea/efeitos adversos , Doença Enxerto-Hospedeiro/etiologia , Histiocitose Sinusal/etiologia , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicações , Adolescente , Antígenos CD , Antígenos de Diferenciação Mielomonocítica , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Evolução Fatal , Histiócitos , Humanos , Masculino , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Indução de Remissão , Proteínas S100RESUMO
Factor VII deficiency is a serious, potentially lethal disorder of blood coagulation caused by a defect in hepatic factor VII synthesis. We report two children, sisters, both with severe, recurrent hemorrhagic complications from factor VII deficiency, successfully treated with orthotopic liver transplantation. Postoperatively, they remain symptom free with normal coagulation profiles.
