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1.
Bol Asoc Med P R ; 104(3): 41-6, 2012.
Artigo em Inglês | MEDLINE | ID: mdl-23156891

RESUMO

Chronic myeloid neoplasm with eosinophilia and abnormality of platelet-derived growth factor receptor alpha (PDGFRA), referred as chronic eosinophilic leukemia, is an extremely rare neoplasm where long-term prognosis is uncertain though a high grade of responsiveness to Imatinib has been reported. The mortality and morbidity associated with chronic eosinophilic leukemia is associated with the degree of tissue involvement, damage, or both at diagnosis. We discuss a case of a young male patient with past medical history of hypoglycemia that presented to the emergency room with a complaints of a sharp abdominal pain localized in the upper quadrants. Laboratories were remarkable for elevated white blood cells with eosinophils predominance, anemia and thrombocytopenia. Bone marrow biopsy dislocated a FIP1L1-PDGFRA fusion gene chronic eosinophilic leukemia. Physicians need to have a high index of suspicion of this rare entity since not all eosinophilias can be interpreted as asthma or parasitis infections.


Assuntos
Síndrome Hipereosinofílica/complicações , Leucemia Mieloide/complicações , Receptor alfa de Fator de Crescimento Derivado de Plaquetas , Asma , Árvores de Decisões , Humanos , Masculino , Doenças Parasitárias , Adulto Jovem
2.
Biol Blood Marrow Transplant ; 15(6): 656-61, 2009 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-19450749

RESUMO

Treatment of patients with paroxysmal nocturnal hemoglobinuria (PNH) has been traditionally empirical, primarily aiming at ameliorating symptoms or treating complications resulting from the disease. Novel therapies such as eculizumab result in stabilization of hemoglobin levels and improvement in quality of life, but does not cure PNH. Nonrandomized studies suggest that long-term remissions are achievable when using myeloablative or nonmyeloablative/reduced-intensity (NMT/RIC) allogeneic hematopoietic stem cell transplantation (HSCT) as treatment for PNH. Nevertheless, patients with previous life-threatening complications from PNH may be more appropriately treated with an NMT/RIC regimen, rather than a myeloablative approach, because of the increased transplant mortality associated with the latter. The decision to perform an allogeneic HSCT (allo-HSCT) should weigh disease prognosis, by incorporating known adverse prognostic factors such as previous history of thrombosis and/or evolution to pancytopenia, among others, against the risk of transplant-related complications. Selection of the appropriate candidate and, equally important, the right time to perform an allo-HCT are important questions that need to be answered in the context of large prospective randomized trials.


Assuntos
Transplante de Células-Tronco Hematopoéticas , Hemoglobinúria Paroxística/cirurgia , Adolescente , Adulto , Anticorpos Monoclonais/uso terapêutico , Anticorpos Monoclonais Humanizados , Anticoagulantes/uso terapêutico , Transfusão de Sangue , Antígenos CD55/análise , Antígenos CD59/análise , Criança , Ensaios Clínicos como Assunto/estatística & dados numéricos , Danazol/uso terapêutico , Glucocorticoides/uso terapêutico , Transplante de Células-Tronco Hematopoéticas/mortalidade , Hemoglobinúria Paroxística/diagnóstico , Hemoglobinúria Paroxística/tratamento farmacológico , Hemoglobinúria Paroxística/terapia , Humanos , Pessoa de Meia-Idade , Agonistas Mieloablativos/administração & dosagem , Agonistas Mieloablativos/efeitos adversos , Complicações Pós-Operatórias/prevenção & controle , Prognóstico , Indução de Remissão , Fatores de Risco , Trombofilia/etiologia , Condicionamento Pré-Transplante/métodos , Condicionamento Pré-Transplante/mortalidade , Transplante Homólogo , Adulto Jovem
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