Assuntos
Astenia/complicações , Basófilos/patologia , Leucemia Basofílica Aguda/complicações , Leucemia Basofílica Aguda/patologia , Idoso , Antibióticos Antineoplásicos/uso terapêutico , Antígenos CD/análise , Antimetabólitos Antineoplásicos/uso terapêutico , Azacitidina/uso terapêutico , Medula Óssea/patologia , Citarabina/uso terapêutico , Feminino , Humanos , Idarubicina/uso terapêutico , Leucemia Basofílica Aguda/sangue , Leucemia Basofílica Aguda/tratamento farmacológico , Leucemia Mieloide Aguda/complicações , Leucemia Mieloide Aguda/tratamento farmacológicoRESUMO
Patients with 47, XXY karyotype (Klinefelter syndrome) appear to have increased risk of developing cancer, especially male breast cancer, germ cell tumours and non Hodgkin lymphomas, but rarely acute myeloid leukaemia. We report a patient with acute basophilic leukaemia with 47, XXY karyotype in both the tumour and constitutional cells. Acute basophilic leukaemia is very rare disease comprising less than 1% of all acute myeloid leukaemias. Morphological characteristic of leukaemic blast cells is moderately basophilic cytoplasm containing a variable number of coarse basophilic granules. The most characteristic cytochemical reaction is metachromatic positivity with toluidine blue. Blast are myeloperoxidase negative. Also leukemic blasts express myeloid and monocyte markers. There is no consistent chromosomal abnormality identified in this leukaemia. This is the first reported case of acute basophilic leukaemia in patient with Klinefelter syndrome. In this article the medical history of the patient is given and the possible connection between Klinefelter syndrome and acute myeloid leukaemia is discussed.
Assuntos
Síndrome de Klinefelter/complicações , Leucemia Basofílica Aguda/complicações , Crise Blástica , Células da Medula Óssea/patologia , Aberrações Cromossômicas , Mapeamento Cromossômico , Evolução Fatal , Humanos , Cariotipagem , Síndrome de Klinefelter/genética , Síndrome de Klinefelter/patologia , Leucemia Basofílica Aguda/genética , Leucemia Basofílica Aguda/patologia , Masculino , Pessoa de Meia-Idade , Insuficiência de Múltiplos Órgãos/patologiaAssuntos
Histiocitose de Células de Langerhans/complicações , Leucemia Basofílica Aguda/complicações , Adulto , Antígenos CD/análise , Antígenos CD1/análise , Antígenos de Diferenciação Mielomonocítica/análise , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Biomarcadores , Medula Óssea/ultraestrutura , Transplante de Medula Óssea , Grânulos Citoplasmáticos/ultraestrutura , Evolução Fatal , Histiocitose de Células de Langerhans/metabolismo , Histiocitose de Células de Langerhans/patologia , Histiocitose de Células de Langerhans/cirurgia , Humanos , Quimioterapia de Indução , Leucemia Basofílica Aguda/tratamento farmacológico , Leucemia Basofílica Aguda/patologia , Leucemia Basofílica Aguda/cirurgia , Masculino , Células-Tronco Neoplásicas/ultraestrutura , Complicações Pós-Operatórias , Proteínas S100/análise , Pele/química , Pele/patologiaAssuntos
Basófilos/patologia , Doenças Cardiovasculares/etiologia , Leucemia Basofílica Aguda/diagnóstico , Proteínas Proto-Oncogênicas c-ets/genética , Proteínas Repressoras/genética , Idoso de 80 Anos ou mais , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Exame de Medula Óssea , Doenças Cardiovasculares/diagnóstico , Doenças Cardiovasculares/terapia , Eletrocardiografia , Evolução Fatal , Feminino , Humanos , Hibridização in Situ Fluorescente , Leucemia Basofílica Aguda/complicações , Leucemia Basofílica Aguda/tratamento farmacológico , Leucemia Basofílica Aguda/genética , Leucemia Basofílica Aguda/patologia , Insuficiência de Múltiplos Órgãos/etiologia , Insuficiência Respiratória/etiologia , Resultado do Tratamento , Variante 6 da Proteína do Fator de Translocação ETSRESUMO
BACKGROUND: Acute de novo basophilic leukemia (ABL) is uncommon in adults, and extremely rare in children. To the authors' knowledge, there have been no previous reports of anaphylactoid reactions from basophilic degranulation in children with this condition. METHODS: This report describes the clinicopathologic profile and complications of a patient with de novo ABL. RESULTS: Immediately after the first induction dose of intravenous vincristine, the patient developed an anaphylactoid reaction and disseminated intravascular coagulation with massive pulmonary hemorrhage. A normal serum tryptase level suggested that this life-threatening event was secondary to tumor lysis (basophilic degranulation), rather than to a mast-cell mediated anaphylactic reaction to vincristine. This interpretation is supported by the coagulation studies, which suggested release of heparin from the blast granules. CONCLUSIONS: Although de novo ABL is rare, it should be considered when cytoplasmic basophilic granules are seen in the leukemic cells of patients with what otherwise appears to be undifferentiated leukemia, and the pertinent diagnostic procedures should be undertaken. During the treatment of ABL, potential complications related to basophilic degranulation should be anticipated, and antihistamine prophylaxis may be of value.
Assuntos
Anafilaxia/induzido quimicamente , Coagulação Intravascular Disseminada/etiologia , Leucemia Basofílica Aguda/complicações , Vincristina/efeitos adversos , Degranulação Celular , Feminino , Humanos , Lactente , Leucemia Basofílica Aguda/tratamento farmacológico , Leucemia Basofílica Aguda/patologiaRESUMO
Acute basophilic leukaemia is usually characterized by a very rapid clinical course, hyperhistaminemia, resistance to antineoplastic therapy and early death due to complications related to disease. This entity is a rare condition, accounting for less than two percent of all haematopoietic malignancies. Most of the case reports are basophilic blast crisis in patients with a previous lympho or myeloproliferative disorder. A 62-year-old woman who was diagnosed as Philadelphia positive chronic myelogenous leukaemia after four years of evolution developed a basophilic blast crisis, whose characteristics are reported. Accompanying this transformation there was also a cytogenetic change. Despite chemotherapy the patient died of disease progression.