Search

Introduction: Sickle cell emergencies are the most common cause of hospitalization for patients with sickle cell disease (SCD). Hospital readmissions represent a considerable financial burden for healthcare systems and increase patient morbidity and mortality. The aim of this study was to assess the prevalence and predictive factors of sickle cell emergencies readmission. Materials and Methods: We conducted a prospective, cross‐sectional, descriptive, and analytical study over a 4‐month period, including all adult patients admitted for an emergency related to SCD: vaso‐occlusive crisis (VOC), acute chest syndrome (ACS), severe anemia, infections, priapism, and stroke. Readmission was considered when the patient returned to the emergency within a period of <30 days, either due to recurrence, persistence of the same complication, or the occurrence of another acute complication related to SCD. Results: We recorded 151 sickle cell emergencies for 112 patients, representing 0.33 emergencies/month/patient. Fifty‐eight cases of readmission were recorded, resulting in a readmission rate of 38.41%. Among these patients, 53 (91.37%) had two admissions, and 5 (8.62%) had three admissions. The median age of the patients was 28.41 years (16–70 years), and the sex ratio was 0.57. The SS sickle cell phenotype was predominant in 97 patients (86.61%). The reasons for readmission were VOC (82.75%), ACS (13.72%), and severe anemia (3.44%). The main factors that predicted readmission were the existence of professional activity, a low fetal hemoglobin (HbF) level, the existence of neutrophilia, lymphocytosis, and/or thrombocytosis (p values of 0.0084, 0.043, and 0.020 respectively). Conclusion: The 30‐day readmission rate after a sickle cell emergency is high in our study. The main factors that predicted readmission were the existence of professional activity, a relatively low level of fetal Hb, the existence of neutrophilia, lymphocytosis, and/or thrombocytosis. [ABSTRACT FROM AUTHOR]

Summary: The degree of anaemia in sickle cell disease (SCD) is a well‐known contributor to morbidity and mortality. We aimed to explore the factors affecting haemoglobin (Hb) level in African SCD patients, considering haemolysis biomarkers (LDH and bilirubin level, and reticulocyte count), leucocyte and platelet counts and socio‐demographic characteristics (gender, age group, country of residence and BMI). The research was part of the CADRE multinational cohort and involved 3699 SCD patients living in Mali, Senegal, Ivory Coast, Democratic Republic of Congo, Gabon and Cameroon: 2936 SS/Sβ0, 587 SC and 176 Sβ + patients with median Hb level of 8, 11.3 and 11.2 g/dL respectively (p < 0.001). In multivariate analysis conducted in 1394 SS/Sβ0 patients, living in Cameroon, female gender, lower BMI, higher haemolysis markers (especially LDH) and higher leucocyte and platelet counts were independently associated with lower Hb level (all p < 0.05). In 497 SC and 156 Sβ + patients, female gender (p < 0.001), lower BMI (p < 0.05) and higher platelet counts (p < 0.001) were independently associated with lower Hb level. Anaemia in African SCD patients is not only associated with haemolysis but also with the country of residence, lower BMI and leucocyte or platelet counts which might reflect inflammation related to infectious burden in the region. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Objectives. The aim of this study was to describe the morbidity and mortality of homozygous sickle cell disease after the age of 40. Methods. This was a cohort study of 209 patients followed from 1994 to 2022. All hemoglobin electrophoresis-confirmed SS sickle cell patients over 40 years were included. A descriptive study of epidemiological, diagnostic, therapeutic, and evolutionary data was used to assess morbidity and mortality. Results. Sex ratio (M/F) was 0.6. Median age was 47 (41–75). According to morbidity, 95.1% had less than 3 vaso-occlusive crises/year. Acute anemia was the most frequent complication (52.63%). Chronic complications were noted in 32.5%. At diagnosis, mean hemoglobin was 8.1 g/dl ± 1.9, HbS was 86.5 ± 10, and HbF was 9.4 ± 7.6. Number of patients transfused was 66%. We noted that 8.1% of patients died, 29.2% were lost to follow-up, and 62.7% were still being followed up. The risk factors identified for death were geographical origin, comorbidity, high HbS, low HbF, and thrombocytosis. Conclusion. This study shows that homozygous SCD is increasingly becoming an adult disease and that it can be carried into old age in Africa. Advanced age over 40 is marked by an upsurge in chronic complications, making it essential to set up a screening program and to organize multidisciplinary follow-up. [ABSTRACT FROM AUTHOR]

Key Clinical Message RDD (Rosai Dorfman disease) is a rare and benign histiocytic proliferative disorder of unknown etiology. FNAC (Fine-needle aspiration cytology) is a useful and reliable tool for the diagnosis of RDD, and as such, biopsy is avoidable. [ABSTRACT FROM AUTHOR]