BACKGROUND: Hemoglobin sickle cell disease is one of the most frequent hemoglobinopathies. Surprisingly, few studies have been dedicated to this disease, currently considered to be a mild variant of homozygous sickle cell disease. The aim of this study was to update our knowledge about hemoglobin sickle cell disease. DESIGN AND METHODS: The study involved a single center series of 179 patients. Clinical and biological data were collected with special attention to the assessment of pulmonary arterial hypertension and nephropathy. RESULTS: Hemoglobin sickle cell diagnosis was delayed and performed in adulthood in 29% of cases. Prevalence of hospitalized painful vasoocclusive crisis, acute chest syndrome and priapism was 36%, 20% and 20%, respectively. The most common chronic organ complications were retinopathy and sensorineural otological disorders in 70% and 29% of cases. Indeed, prevalence of complications reported in homozygous sickle cell disease, such as nephropathy, suspicion of pulmonary hypertension, strokes and leg ulcers was rather low (13%, 4% and 1%, respectively). Phlebotomy performed in 36% of this population (baseline hemoglobin 11.5 g/dL) prevented recurrence of acute events in 71% of cases. CONCLUSIONS: Our data suggest that hemoglobin sickle cell disease should not be considered as a mild form of sickle cell anemia but as a separate disease with a special emphasis on viscosity-associated otological and ophthalmological disorders, and with a low prevalence of vasculopathy (strokes, pulmonary hypertension, ulcers and nephropathy). Phlebotomy was useful in reducing acute events and a wider use of this procedure should be further investigated.
BACKGROUND: Hemoglobin sickle cell disease is one of the most frequent hemoglobinopathies. Surprisingly, few studies have been dedicated to this disease, currently considered to be a mild variant of homozygous sickle cell disease. The aim of this study was to update our knowledge about hemoglobin sickle cell disease. DESIGN AND METHODS: The study involved a single center series of 179 patients. Clinical and biological data were collected with special attention to the assessment of pulmonary arterial hypertension and nephropathy. RESULTS: Hemoglobin sickle cell diagnosis was delayed and performed in adulthood in 29% of cases. Prevalence of hospitalized painful vasoocclusive crisis, acute chest syndrome and priapism was 36%, 20% and 20%, respectively. The most common chronic organ complications were retinopathy and sensorineural otological disorders in 70% and 29% of cases. Indeed, prevalence of complications reported in homozygous sickle cell disease, such as nephropathy, suspicion of pulmonary hypertension, strokes and leg ulcers was rather low (13%, 4% and 1%, respectively). Phlebotomy performed in 36% of this population (baseline hemoglobin 11.5 g/dL) prevented recurrence of acute events in 71% of cases. CONCLUSIONS: Our data suggest that hemoglobin sickle cell disease should not be considered as a mild form of sickle cell anemia but as a separate disease with a special emphasis on viscosity-associated otological and ophthalmological disorders, and with a low prevalence of vasculopathy (strokes, pulmonary hypertension, ulcers and nephropathy). Phlebotomy was useful in reducing acute events and a wider use of this procedure should be further investigated.
Authors: F Schmitt; F Martinez; G Brillet; I Giatras; G Choukroun; R Girot; D Bachir; F Galacteros; B Lacour; J P Grünfeld Journal: Am J Kidney Dis Date: 1998-08 Impact factor: 8.860
Authors: O S Platt; D J Brambilla; W F Rosse; P F Milner; O Castro; M H Steinberg; P P Klug Journal: N Engl J Med Date: 1994-06-09 Impact factor: 91.245
Authors: H F Bunn; C T Noguchi; J Hofrichter; G P Schechter; A N Schechter; W A Eaton Journal: Proc Natl Acad Sci U S A Date: 1982-12 Impact factor: 11.205
Authors: Paulo V Rezende; André R Belisário; Érica L Oliveira; Jéssica A Almeida; Larissa M M Oliveira; Maristela B S R Muniz; Marcos B Viana Journal: Haematologica Date: 2019-01-10 Impact factor: 9.941
Authors: Ana Carolina Cabañas-Pedro; Josefina A P Braga; Roberta F Camilo-Araújo; Ana I M Silva; Perla Vicari; Maria Figueiredo Journal: Haematologica Date: 2013-01 Impact factor: 9.941