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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]

Background/aim: The evolution of sickle cell disease (SCD) is marked by the occurrence of painful episodes linked to the obstruction of microvessels by sickle cells, known as vaso-occlusive crisis (VOC). The aim of this work was to report the practical aspects of the management of acute pain in adults with SCD. Recommendations based on these practices are also provided. Materials and methods: This prospective, cross-sectional, descriptive, and analytical study was conducted over a four-month period of all sickle cell patients admitted to emergency departments for VOC. The parameters studied were sociodemographic, clinicobiological, therapeutic, and evolutionary. Results: There were 118 cases of VOC identified, representing a prevalence of 78.14% of sickle cell emergencies. The mean age of the patients was 28.41 years. The SS sickle cell phenotype accounted for 86.61% of the cases. Osteoarticular pain was the reason for admission for 88.39% of the patients; it was located in the lower limbs in 39.08% and in the spine in 27.1%. Pain intensity was moderate in 6.25% of the patients, intense in 31.25%, and unbearable in 55.55%. Multimodal analgesia was the most commonly used treatment method, combining those of levels one and two (74.31%) and levels one and three (8.25%). The mean dose of morphine administered was 17.14 mg when morphine alone was prescribed for titration, 13.57 mg when paracetamol and morphine were combined, and 15.83 mg when nefopam and morphine were combined. Clinical outcome was favorable in 68.87% of the cases. Conclusion: Wide variability was observed in the modalities of analgesic treatment of sickle cell VOC. These variations reflect different views on the appropriateness of opioids. This study highlights the efficacy of multimodal analgesia in the management of acute pain in patients with SCD, particularly in regard to morphine sparing. Context-specific recommendations will be needed to harmonize practices. [ABSTRACT FROM AUTHOR]

The Hodgkin variant Richter syndrome (HvRS) is an infrequent complication occurring in 1% of lymphocytic lymphoma/chronic lymphocytic leukemia patients. We report a case of HvRS diagnosed in Sub-Saharan Africa. A 63-year-old patient was consulted for the investigation of an abdominal mass that had been evolving for 5 years prior to admission. His history revealed night sweats, 13% weight loss in 3 months and persistent pruritis. Examination revealed bilateral cervical axillary and inguinal macroadenopathies, painless abdominal distension, pruritic lesions and WHO 2 PS. The blood count showed anemia at 9.5 g/dL. Histology revealed a lymphomatous proliferation of diffuse architecture, nodular in places, with Hodgkin and Sternberg cells associated with small lymphocytes, histiocytes and eosinophilic polymorphs. Immunohistochemistry showed CD20, PAX5, BCL2, CD5, CD23 and MYC positivity; Ki67 at 10% and cyclin D1, BCL6 and CD10 negativity; CD30 positivity on Hodgkin and Sternberg cells that remained CD20 negative; difficulty interpreting CD15; EBV positivity (EBERs); and CD3 and CD5 positivity on reactive T cells. CD138 and kappa and lambda light chains were non-contributory. The extension work-up classified the patient as Ann Arbor stage III B with a Hasenclever score of 3/7. This case illustrates the difficulties in diagnosing HvRS in our countries, where the number of haematopathologists is insufficient and the technical facilities are limited. [ABSTRACT FROM AUTHOR]

SUMMARY: COVID-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. [ABSTRACT FROM AUTHOR]

The 2022 World Federation of Haemophilia Annual Global Survey (AGS) reports that 454,690 patients with inherited bleeding disorders (IBD) have been identified globally. While this represents noteworthy progress, haemophilia epidemiology data indicate that 75% of people with inherited bleeding disorders living in low‐income and low‐to‐middle‐income countries have yet to be diagnosed. The AGS also revealed that 11 billion clotting factor units are available to treat haemophilia A and B globally. Due to a lack of finance, these treatments are unavailable to haemophilia in low‐income countries with a consequence lack of access equity for haemophilia treatment in these communities. This sobering reality is not limited to haemophilia but applies to von Willebrand Disease (VWD). While VWD is the most prevalent IBD, only 103,844 people living with this condition have been diagnosed globally. Of the diagnosed patients, only a fraction live in low‐ or middle‐income countries. Moreover, the majority of VWD patients are still treated sub‐optimally without replacement therapies or prophylaxis, both of which are now accepted as global standards of care. In this state‐of‐the‐art review, the authors reflect on three issues. First, the minimum elements required to diagnose haemophilia in a resource‐constrained setting are identified. Second, this review points to the critical stakeholders and outlines their roles in removing access to haemophilia treatment barriers. Finally, the authors examine von Willebrand disease's ongoing diagnostic and treatment challenges and compare these to haemophilia. With the rapidly evolving novel therapies, the therapeutic landscape of all IBD will likely change for the better. [ABSTRACT FROM AUTHOR]

BackgroundSub-Saharan Africa remains the epicenter of the human immunodeficiency virus (HIV) pandemic. However, there is a lack of multicenter data on the risk of transfusion-transmitted HIV from blood centers in sub-Saharan Africa.Study design and methodsThe incidence of HIV infections in the blood donations collected in the main blood banks of five countries (Burkina Faso, Congo, Ivory Coast, Mali, and Senegal) was determined to estimate the current transfusion risk of HIV infection using the incidence rate/window period model.ResultsThe risk of transfusion-transmitted HIV infections associated with the window period varied from 1 in 90,200 donations (Senegal) to 1 in 25,600 (Congo). Considering the five participating blood centers as a whole, the incidence rate of HIV-positive donors per 100,000 person-years was 56.6 (95% confidence interval [CI], 47.1-67.9); the residual risk (RR) was 34.1 (95% CI, 7.8-70.7) per 1 million donations, which represents 1 in 29,000 donations (95% CI, 1/128,000-1/14,000).ConclusionRR estimates varied according to the country. This is potentially due to a lower incidence of HIV infection in the general population or to a more efficient selection of blood donors in the countries with the lowest risk. The estimates of the transfusion risk of HIV infection in each country are important, both to assess the impact of current preventative strategies and to contribute data to policy decisions to reinforce transfusion safety.

Background and Objectives: Plasma‐derived medicinal products (PDMPs) are essential to treat many chronic conditions such as haemophilia and primary immunodeficiency. Patients living in low middle‐income and low‐income countries (LMICs and LICs, respectively) have limited access to PDMPs. The aim of this article is to explore the challenges of accessing PDMPs in LMICs and LICs. Materials and Methods: A review of the literature and reports on blood safety, plasma production and its utilization to produce PDMPs in LMICs and LICs was carried out. Results: There is huge wastage of recovered plasma in LMICs and LICs as a result of a lack of good manufacturing practice (GMP) in the production of plasma for fractionation. Together with the high cost of imported PDMP procurement, patients have limited access to such products. Conclusion: There is a need to improve the situation by using domestically sourced plasma through the initiation of local plasma programmes through a stepwise approach to improve access to PDMPs in LMICs and LICs. [ABSTRACT FROM AUTHOR]