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Background and ObjectivesInflammatory markers that influence bone pain crisis (BPC) and other complications of sickle cell anaemia (SCA) are numerous and play various roles. This study determined the plasma levels of tumour necrosis factor (TNF) - α, interleukin - 8 (IL-8), and endothelin - 1 (ET-1) in adult SCA patients during BPC and in steady state. In addition, the plasma levels of these cytokines were correlated with the severity of BPC of the patients.Methods and MaterialsSixty adult SCA patients (30 during BPC and 30 during steady state) and 30 haemoglobin A controls were enrolled for this cross-sectional study. The severity of BPC was assessed clinically, and questionnaires were filled. Plasma levels of TNF- α, IL-8 and ET-1 were quantified by ELISA, and haematological parameters were determined using a 5-part auto-analyzer. Plasma levels were correlated with the severity of bone pain crisis. Results were considered statistically significant if p<0.05.ResultsPlasma TNF-α, IL-8, and ET-1 were significantly elevated in the BPC group than in the steady state group and the controls. Plasma TNF-α, IL-8 and ET-1 were markedly higher in the severe BPC groups than the steady state and control groups, There was a positive correlation between TNF-α and ET-1 in the bone pain crisis group.ConclusionElevated levels of plasma TNF-α, IL-8, and ET-1 further establish the chronic inflammatory state in SCA and equally affirm their significant contribution, not only to pathogenesis but also to the severity of pain in SCA.

This case highlights the fact that bone involvement is the commonest clinical manifestation of Sickle Cell Disease (SCD) both in the acute settings such as painful vaso-occlusive crisis (VOC) and as a source of chronic, progressive debility such as avascular necrosis (AVN), chronic osteomyelitis and fixed flexion deformity of joints.Protracted multiple bone involvement i.e. bilateral femoral and left humeral chronic osteomyelitis, Left elbow, Left knee and right humeral septic arthritis together with avascular necrosis of both femoral and right humeral heads, coupled with urinary tract infection (UTI) and decubitus ulcer in a young adult SCD patient is an unusual occurrence. This morbidities resulted into an uninterrupted 29 weeks of hospitalization for the patient who had previously enjoyed fairly good health.Various micro-organisms were sequentially cultured at various times and sites; these include E coli and Klebsiella in urine and klebsiella spp in the aspirates of the affected knee joint, elbow joint and femoral osteomyelitis. A screen for HIV 1 and 2 were non-reactive.Multidisciplinary approach was applied to the patient who was finally discharged home on a wheelchair. This case reflects not only the high susceptibility of SCD patients to infection, but also the morbidity and the attendant complications. It also highlights the need to forestall VOC which often predisposes to osteomyelitis. There is a need to have a highly organized, well-equipped and highly subsidized Sickle Cell and rehabilitation center in Nigeria in order to improve the medical care for SCD patients.

Thrombocytopenia absent radius (TAR) syndrome is a rare congenital disorder that is consistently associated with skeletal abnormality and thrombocytopenic haemorrhage. This is a case of a neonate with bilateral absent radius and thrombocytopenia. The rarity of this case prompted this report.

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Background: Brazil became the South American epicenter for coronavirus disease (COVID-19) soon after the first case was diagnosed in February 2020 with the highest infection rate occurring in the state of Sao Paulo. COVID-19 is characterized by marked thrombo-inflammation mechanisms, and neutrophil-lymphocyte ratio (NLR) among many clinical and laboratory data, is becoming an inflammatory marker of severity and mortality of COVID-19. We evaluated the serial weekly lymphocyte ratios, which are easily derivable from the routine blood counts, in the survivors and non-survivors of COVID-19 at the Clinical Hospital of University of Campinas (UNICAMP), Campinas, Sao Paulo, Brazil, from time of diagnosis to the 3 rd week of care. This hospital is one of the referral centers for COVID-19 patients in this state. Methods: In this retrospective study, we reviewed the medical notes of 320 adults hospitalized patients with PCR-confirmed COVID-19 at the Clinical Hospital of UNICAMP, from March 2020 to March 2021. The serial weekly hematological parameters (analyzed using automated counter - XN 9000™, Sysmex, Japan) from the time of diagnosis were analyzed and lymphocytes ratios (neutrophil-lymphocyte, NLR, platelet-lymphocyte PLR, and monocyte-lymphocyte MLR) were calculated. The survivors (n=257) were those who recovered from the disease and were discharged from the hospital, while the non-survivors (n=63) were those who died in the course of treatment. Statistical analyses were performed using SPSS (version 22). Unpaired data of Survivors and Non-survivors with COVID-19 were compared using Mann-Whitney tests. Repeated measures were compared within and between groups using univariate and multivariate tests in general linear models. All results were considered significant if p&lt;0.05. Results: Of the 320 patients, 257 (80.3%) were survivors and had lower mean age than the non-survivors (57.73 vs 64.65 years, p&lt;0.001). At diagnosis, the non-survivors had a lower lymphocyte count (p=0.002), basophil count (p=0.049), and hematocrit (p=0.021) than the survivors, Table 1. We used general linear models for repeated measures and corrected for the patients who did not stay long enough to have a complete series of blood counts, Figure 1 A-G. Multivariate tests between the survivor and non-survivor groups showed significant variations with serial weekly lymphocyte count (p&lt;0.001), neutrophil count (P=0.005), NLR (p=0.009), MLR (p=0.010), and PLR (p=0.035) but not with the weekly monocyte count (p=0.352) and platelet count (p=0.505). The NLR was higher and PLR was lower in the non-survivors at diagnosis (p&lt;0.001 and p=0.047 respectively), both were higher in the 2 nd week post-diagnosis (p&lt;0.001 and 0.043 respectively), and in the 3 rd week (p&lt;0.001 and p=0.043 respectively) (Figure 1D and E). The MLR was not significantly different at diagnosis but became elevated in the following two weeks post-diagnosis (p=0.09, p=0.022, and p&lt;0.001 respectively) (Figure 1F). Conclusions: The non-survivors were older and their NLR and MLR tend to increase from the time of diagnosis while their PLR tend to decrease after the 2 nd week post-COVID-19 diagnosis and treatment. On the other hand, all three ratios significantly decrease in the survivors. While neutrophilia and lymphopenia improved in the survivor, they worsen in non-survivors. These cells may have contributed towards the recovery by ameliorating the inflammatory response in survivors, and death by worsening the response in non-survivors of COVID-19. This study shows that serial lymphocyte count, neutrophil count, NLR, PLR, and MLR could serve as good and easily accessible markers of outcomes in patients with COVID-19 and could be used for monitoring of response to treatment. Figure 1 Figure 1. Disclosures Costa: Novartis: Consultancy.