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Patrón clínico y hematológico de pacientes con drepanocitosis o diagnóstico presuntivo de talasemia

Keywords: sickle cell anemia, venezuela, thalassemia, sucre state.

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Abstract:

clinical and hematological characteristics of 14 patients with sickle cell anemia; one heterozygous as, and 7, with diagnostic of microcytic hypochromic anemia were analyzed. hemoglobin phenotypes were identified by electrophoresis, fetal hemoglobin was quantificated for alkaline denaturation and the hba2 for ionic exchange chromatography; -α3,7-thalassemia was detected by mutation identification using polymerase chain reaction (pcr). ss phenotype was confirmed in 10 patients, two were ssf, one was ssfa2, and one was asf (hbf - 2%). the patient diagnosed as as was ssf (hbf = 21%). ad-patients presented a moderate clinical course of the illness. five microcytic hypochromic anemia patients were hbaa, one was hbaaa2 and another hbaaf; those patients present a high hematological and clinical variation, β-thalassemia was 19%. -α3,7 -thalassemia was not detected. infection was most frequent clinical manifestation (respiratory tract infection and intestinal parasitism). these results shows that -α3,7 -thalassemia are not modulator genetic factors of clinical and hematological manifestations of patients with microcytic hypochromic anemia and sickle cell anemia. we suggest that environmental factors such as respiratory tract infection and intestinal parasitism may be affect the course of illness.

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