RESUMEN
Myelodysplastic syndromes (MDS) encompass a heterogeneous group of clonal hematopoietic stem cell disorders characterized by a broad clinical spectrum related to ineffective hematopoiesis leading to unilineage or multilineage cytopenias, with a high propensity for transformation to acute myeloid leukemia. Iron overload has been recently identified as one of the important conditions complicating the management of these diverse disorders. The accumulation of iron is mainly related to chronic transfusions; however, evidence suggests a possible role for ineffective erythropoiesis and increased intestinal absorption of iron, related to altered hepcidin and growth differentiation factor-15 levels in the development of hemosiderosis in patients with MDS. In addition to its suggested role in the exacerbation of ineffective erythropoiesis, multiple reports have identified a prognostic implication for the development of iron overload in patients with MDS, with an improvement in overall survival after the initiation of iron chelation therapy. This review includes a detailed discussion of iron overload in patients with MDS whether they are undergoing supportive therapy or curative hematopoietic stem cell transplantation, with a focus on the mechanism, diagnosis, and effect on survival as well as the optimal management of this highly variable complication.
Asunto(s)
Sobrecarga de Hierro , Síndromes Mielodisplásicos , Anemia/complicaciones , Anemia/terapia , Transfusión Sanguínea/estadística & datos numéricos , Terapia por Quelación/métodos , Humanos , Hierro/efectos adversos , Hierro/metabolismo , Sobrecarga de Hierro/epidemiología , Sobrecarga de Hierro/etiología , Sobrecarga de Hierro/terapia , Leucemia Mieloide Aguda/sangre , Leucemia Mieloide Aguda/epidemiología , Leucemia Mieloide Aguda/etiología , Leucemia Mieloide Aguda/prevención & control , Síndromes Mielodisplásicos/complicaciones , Síndromes Mielodisplásicos/epidemiología , Síndromes Mielodisplásicos/terapia , Trombocitopenia/complicaciones , Trombocitopenia/terapia , Reacción a la Transfusión/terapiaRESUMEN
A 28-year-old Lebanese thalassemia intermedia (TI) patient with homozygous IVS1-110 mutation sustained atypical chest pain of 1 day's duration. The EKG reading revealed ST segment elevation in the chest leads V(1) to V(5). Coronary angiography showed 2 plaques in the left anterior descending coronary artery. He underwent subsequent angioplasty with stenting of the left anterior descending coronary artery. An extensive thrombophilia profile was negative. He was started on medication, and his medical condition improved and chest pain ceased. This is the first case report of myocardial infarction in a TI patient among thalassemics. We propose that such cases will emerge more frequently as our population ages, keeping in mind a possible thrombotic mechanism.
Asunto(s)
Infarto del Miocardio/complicaciones , Trombosis/complicaciones , Talasemia beta/complicaciones , Adulto , Angioplastia Coronaria con Balón , Angiografía Coronaria , Homocigoto , Humanos , Masculino , Infarto del Miocardio/diagnóstico por imagen , Infarto del Miocardio/terapia , Stents , Trombosis/genética , Talasemia beta/genéticaRESUMEN
Venous thrombosis is a well-recognized complication of lenalidomide therapy in patients with multiple myeloma, but its occurrence during the treatment of other hematologic malignancies is less well described. In this report, we detail the occurrence of corpora cavernosa thrombosis in a patient receiving lenalidomide for the therapy of myelofibrosis. This case highlights the need for clinical vigilance in patients who present with unusual symptoms during investigational therapy, and indicates that the occurrence of venous thrombosis complicating therapy with lenalidomide or related compounds is not isolated to patients with multiple myeloma.