RESUMO
The population of Viet Nam, is 96.2 million, of which 13.8% are carriers of thalassemia genes. Thalassemia/hemoglobinopathies carriers exist at different frequencies in all 54 ethnic groups of the country. Gene carrier rate and globin gene mutation rate varies ethnically and topographically. The ethnic groups in the Northern Highland region have high rates of α0- and ß0-thalassemia (α0- and ß0-thal), while those in the Southern Middle region have high rates of α+-thalassemia (α+-thal) and Hb E (or codon 26) (HBB: c.79G>A). The lowest is found in La Hu (0.23%), while the highest is found in Raglai (88.6%). Thalassemia prevention and control programs were introduced using prenatal and neonatal diagnosis for the prevention of new thalassemic births. Most existing thalassemia patients are undergoing supportive treatment with regular blood transfusions and iron chelation. Curative treatment by hematopoietic stem cell transplantation is available but is limited to a minority of the patients.
Assuntos
Hemoglobinopatias , Talassemia alfa , Talassemia beta , Feminino , Genótipo , Hemoglobinopatias/genética , Heterozigoto , Humanos , Recém-Nascido , Mutação , Gravidez , Vietnã/epidemiologia , Talassemia alfa/diagnóstico , Talassemia alfa/epidemiologia , Talassemia alfa/genética , Talassemia beta/diagnóstico , Talassemia beta/epidemiologia , Talassemia beta/genéticaRESUMO
BACKGROUND: Hyperleukocytosis is commonly seen in acute and chronic leukemias. Therapeutic leukocytapheresis using an automatic cell separator can help to achieve prompt leukoreduction to reduce the rate of thrombotic events and early mortality as well as to prevent tumor lysis syndrome. AIM: In this study, we report a single center's experience in managing leukemia patients with therapeutic leukocytapheresis prior to chemotherapy. MATERIALS AND METHODS: Leukocytapheresis procedures were performed in 192 leukemia patients (including acute myeloid leukemia [AML], acute lymphoblastic leukemia [ALL], and chronic myeloid leukemia [CML]) with hyperleukocytosis between January and December 2016. RESULTS: Median % reduction of white blood cell (WBC) count was 30.5% and median % removal efficiency was 46.7% for 75 procedures where the waste bag was sampled. WBC removal efficiency strongly depended on diagnosis (and was 71%, 66%, and 39% for ALL, AML, and CML, respectively). Procedures were generally well tolerated with only 9 out of 192 patients having mild adverse effects. DISCUSSION AND CONCLUSION: In the absence of specific guidelines for the management of hyperleukocytosis, leukocytapheresis in association with chemotherapy should be considered early in clinical practice.