Abstract

VILACHA, Daniella  and  SALAZAR, Raquel. Hematological and clinical profile in sickle cell or thalassemic patients. Rev. invest. clín. [online]. 2006, vol.58, n.2, pp.94-100. ISSN 2564-8896.

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; -α^3,7-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%. -α^3,7 -thalassemia was not detected. Infection was most frequent clinical manifestation (respiratory tract infection and intestinal parasitism). These results shows that -α^3,7 -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.

Keywords : Sickle cell anemia; Venezuela; Thalassemia; Sucre state.

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