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SUMMARYCOVID-19 infection has emerged as a comorbidity that can significantly increase morbidity and mortality in sickle cell patients with ACS (acute thoracic/chest syndrome). The aim of our study was to assess COVID-19-related morbidity and mortality in sickle cell patients with ACS. This was a retrospective, descriptive study of patient records followed over a 36-month period from January 2020 to December 2022. The study was conducted at the national blood transfusion center in Dakar. The sex ratio (M/F) was 0.82. The median age was 26 (17-39) years. The most represented age group was between 21 and 30 years. Factors associated with death were: at baseline, SS genotype, presence of comorbidities (asthma, chronic obstructive pulmonary disease, viral hepatitis B, ischemic heart disease), osteonecrosis of the femoral head, and use of NSAIDs (non-steroidal anti-inflammatory drugs) at diagnosis of COVID-19; at the diagnosis of ACS associated with COVID-19, respiratory distress, hypoxia (Sa02 < 92%), creatininemia >18.5 mg/l, CRP >192 mg/l, lymphopenia; the therapeutic modalities associated with death were: transfusion of RBCs (packed red blood cells) and curative anticoagulation. This study shows that patients with comorbidities and/or chronic complications of sickle cell disease can develop severe forms of ACS associated with COVID 19, leading to death. Other factors linked to death, notably diagnostic and therapeutic, were also identified in the course of this study.
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Rosai-Dorfman disease (RDD) is a benign histiocytic proliferation that results in nodal and extranodal involvements. It is a rare disease, with fewer than 1,000 cases reported in the literature, which explains its lack of knowledge by physicians and the lack of codified therapeutic strategies. We report the case of an 8-year-old girl who presented a rapidly progressive cervical lymph node mass; the diagnosis of RDD was made based on histology and immunohistochemistry. The patient was treated with oral corticosteroids at a dose of 1 mg/kg/d with a favorable outcome and no recurrence after one year of follow-up. This observation illustrates the clinical presentation and diagnosis of this rare clinicopathological entity. The prognosis and treatment options are also discussed.
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CONTEXT AND OBJECTIVES: Blood transfusions (BT) remain a mainstay of therapy for patients with sickle cell disease (SCD) but pose significant clinical challenges. We aim to assess infectious markers, red cell alloimmunization, and iron overload secondary to BT in SCD patients. MATERIALS AND METHODS: This case-control study included 253 SCD (153 SCD-transfused and 100 SCD non-transfused). We evaluated the transfusion practice (modalities, indications), post-transfusion complications (infections, alloimmunization, iron overload), and risk factors of these complications (socio-demographic, clinical, biological). RESULTS: Median age was 28.5 years (5 - 59). The sex ratio was 0.86. Homozygous SCD was the most common (95.3%). Simple BT was performed in 92.8% and transfusion exchange in 18.9%. Transfusion indications were dominated by acute anemia (57.06%) and vaso-occlusive crisis (VOCs) (14%). Red blood cell concentrates (RBCSs) were administered to 93.46%. The median RBCs received per patient was 10 (2 - 48). The prevalence of VHC in SCD-transfused was 1.33% and 2% for VHB. Anti-HIV antibodies were not found. Red cell alloimmunization frequency was 16%. The most common alloantibodies were anti-rhesus (34.19%) and anti-Kell (23.67%). Iron overload was detected in 7.84%. The number of RBCs transfused was the only risk factor for alloimmunization (p = 0.03) and iron overload (p = 0.023). BT frequency was not related to infectious transmission. CONCLUSION: BT therapy is still a risk for SCD polytransfused patients despite advances in blood safety. Although infectious transmission has rare, the risk of alloimmunization and iron overload is high in these patients.