Contributions
Abstract: PB2411
Type: Publication Only
Background
Hemoglobin sickle cell disease is one of the most frequent hemoglobinopathies in Tunisia.The sickle cell syndromes (SCS) representing by patients with homozygosity for HbS, patients with hemoglobin SC(HbSC, combined heterozygosity for hemoglobins S and C) and patients with sickle cell-beta thalassemia, is still a health problem because of their severe clinical manifestations and their complications.
Aims
The objective of this study is to describe the clinical profile, treatment and complications of these patients in the south Tunisian
Methods
Our study is retrospective having interested the patients with SCS diagnosed and taken care in three centers of the university hospital Hedi chaker of Sfax: Pediatrics Department, Pediatrics Emergency Resuscitation (PUR) and the Hematology Department of CHU Hedi Chaker of Sfax for a period of 35 years (from 1979 to 2014). The diagnosis and classification of the type of SCS is based on clinic data, hemogram, hemoglobin electrophoresis and the family survey.
Results
We collected 76 cases of SCS: 44 cases followed in hematology department, 23 cases in pediatric center and 9 patients in pediatric intensive care unit. The mean age at diagnosis was 8 years (extremes: 1month - 49 years). Sixty-five of our patients (85.5%) were white, 11 (14.5%) were black. Inbreeding (consanguinity) was observed in 37 cases (48.7%). SS homozygous sickle cell disease was noted in 68.4%, hemoglobin SC in one patient (1.3%) and Sß thalassemia in 9 patients.SCS diagnosis was made following anemic syndrome in 46% of cases, a vasoocclusive crisis in 34% of cases, a complication in 4% of cases and hand-foot syndrome in 1.3% of cases.The main acute complications observed in our patients were vaso-occlusive attacks (67%),
infections (44.2%) and aggravation of anemia (24.7%). chronic complications were vesicular lithiasis in 20 patients with osteonecrosis of the femoral head in 5 patients.The symptomatic treatment consisted of antibioticprophylaxis in 85% of the patients, hyperhydration performed in 47 cases (hospitalized for vaso-occlusive attacks), blood transfusion in 40 patients and transfusion exchange in 11 patients(a preoperative preparation in 6 cases,pregnant women in 2 cases and a serious vasoocclusive accident in 3 patients.the background treatment consisted of a transfusion exchange program in 9 patients and Hydroxyurea in 17 patients.
Conclusion
Homozygous sickle cell disease was the most common type of sickle cell disease in our series (68.4%), as also described in most of the series in the literature. The diagnosis of major sickle cell syndrome remains relatively late in our series (8 year) compared to literature. The frequency of vaso-occlusive seizures is the same as that reported in the series, while the infections are a little less due to antibiotic prophylaxis.The rate of anemia, however, remains more important than in the series. Vesicular lithiasis was the most frequently described chronic complication in our study, a little more common than in the literature. Background treatment has improved treatment outcomes and decreased the incidence of vasoconstriction and hospitalization. The bone marrow allograft, not made in our patients remains a pillar in the process that should be introduced in our institution.
Session topic: 27. Sickle cell disease
Keyword(s): Clinical data, Complications, sickle cell disease, Treatment
Abstract: PB2411
Type: Publication Only
Background
Hemoglobin sickle cell disease is one of the most frequent hemoglobinopathies in Tunisia.The sickle cell syndromes (SCS) representing by patients with homozygosity for HbS, patients with hemoglobin SC(HbSC, combined heterozygosity for hemoglobins S and C) and patients with sickle cell-beta thalassemia, is still a health problem because of their severe clinical manifestations and their complications.
Aims
The objective of this study is to describe the clinical profile, treatment and complications of these patients in the south Tunisian
Methods
Our study is retrospective having interested the patients with SCS diagnosed and taken care in three centers of the university hospital Hedi chaker of Sfax: Pediatrics Department, Pediatrics Emergency Resuscitation (PUR) and the Hematology Department of CHU Hedi Chaker of Sfax for a period of 35 years (from 1979 to 2014). The diagnosis and classification of the type of SCS is based on clinic data, hemogram, hemoglobin electrophoresis and the family survey.
Results
We collected 76 cases of SCS: 44 cases followed in hematology department, 23 cases in pediatric center and 9 patients in pediatric intensive care unit. The mean age at diagnosis was 8 years (extremes: 1month - 49 years). Sixty-five of our patients (85.5%) were white, 11 (14.5%) were black. Inbreeding (consanguinity) was observed in 37 cases (48.7%). SS homozygous sickle cell disease was noted in 68.4%, hemoglobin SC in one patient (1.3%) and Sß thalassemia in 9 patients.SCS diagnosis was made following anemic syndrome in 46% of cases, a vasoocclusive crisis in 34% of cases, a complication in 4% of cases and hand-foot syndrome in 1.3% of cases.The main acute complications observed in our patients were vaso-occlusive attacks (67%),
infections (44.2%) and aggravation of anemia (24.7%). chronic complications were vesicular lithiasis in 20 patients with osteonecrosis of the femoral head in 5 patients.The symptomatic treatment consisted of antibioticprophylaxis in 85% of the patients, hyperhydration performed in 47 cases (hospitalized for vaso-occlusive attacks), blood transfusion in 40 patients and transfusion exchange in 11 patients(a preoperative preparation in 6 cases,pregnant women in 2 cases and a serious vasoocclusive accident in 3 patients.the background treatment consisted of a transfusion exchange program in 9 patients and Hydroxyurea in 17 patients.
Conclusion
Homozygous sickle cell disease was the most common type of sickle cell disease in our series (68.4%), as also described in most of the series in the literature. The diagnosis of major sickle cell syndrome remains relatively late in our series (8 year) compared to literature. The frequency of vaso-occlusive seizures is the same as that reported in the series, while the infections are a little less due to antibiotic prophylaxis.The rate of anemia, however, remains more important than in the series. Vesicular lithiasis was the most frequently described chronic complication in our study, a little more common than in the literature. Background treatment has improved treatment outcomes and decreased the incidence of vasoconstriction and hospitalization. The bone marrow allograft, not made in our patients remains a pillar in the process that should be introduced in our institution.
Session topic: 27. Sickle cell disease
Keyword(s): Clinical data, Complications, sickle cell disease, Treatment