RESUMO
A case of Kaposi's sarcoma in a child with no serologic evidence of human immunodeficiency virus (HIV) infection is reported. A 7-year-old boy with Stage IV non-Hodgkin's lymphoma, after conventional chemotherapy, underwent autologous bone marrow transplantation (ABMT). Five months later he presented with supraclavicular mass and mediastinal enlargement. A bone marrow biopsy showed hypoplasia with no signs of the underlying disease, whereas the excised mass revealed a typical histologic pattern of Kaposi's sarcoma. The child is currently being treated with recombinant alpha-interferon (alpha-IFN) and regression of the disease has been achieved.
Assuntos
Transplante de Medula Óssea/efeitos adversos , Leucemia-Linfoma Linfoblástico de Células Precursoras/cirurgia , Sarcoma de Kaposi/etiologia , Criança , Clavícula , Humanos , Masculino , Sarcoma de Kaposi/patologiaAssuntos
Transplante de Medula Óssea/métodos , Histiocitose de Células não Langerhans/cirurgia , Transplante de Medula Óssea/imunologia , Feminino , Antígenos HLA/imunologia , Células-Tronco Hematopoéticas/imunologia , Histiocitose de Células não Langerhans/genética , Histiocitose de Células não Langerhans/imunologia , Histocompatibilidade/imunologia , Humanos , Lactente , Masculino , Transplante HomólogoRESUMO
Severe combined immunodeficiency disease (SCID) with adenosine deaminase (ADA) deficiency is a genetic autosomic recessive disorder with profound impairment of T-cell function, invariably complicated by fatal infections. The absence of ADA-enzyme and the accumulation of deoxy-ATP, with toxic effects on the T-lymphocytes is the common feature of this disease. As a consequence, lymphoid precursors failure to develop into mature T-cells, resulting in absolute lymphopenia and atrophy of the thymus. Bone marrow transplantation from an HLA-identical donor is considered the treatment of choice for this disease. We describe the case of a 1-month-old child with ADA deficiency SCID who underwent bone marrow transplantation (BMT) using paternal haploidentical, lectin-separated marrow, as a source of hemopoietic stem cells.
