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BACKGROUND: Neutrophil-lymphocyte ratio (NLR) and Platelet-Lymphocyte ratio (PLR) are emerging markers of systemic inflammation in chronic disorders, including hemoglobinopathies. OBJECTIVE: This study determined the pattern of NLR and PLR in sickle cell anemia (SCA) patients during steady state and vasoocclusive crisis (VOC). MATERIALS AND METHODS: A cross-sectional study composed of 60 adult SCA patients (30 during VOC and 30 during steady state). Thirty apparently healthy hemoglobin A (HbA) age- and sex-matched controls were included in the study. Complete blood count (CBC) was done for each participant; NLR was calculated as absolute neutrophil count (ANC) divided by the absolute lymphocyte count (ALC), and PLR was calculated as platelet count divided by ALC. STATISTICAL ANALYSIS: Data were analyzed using SPSS (version 22), and the results were considered statistically significant if value of P < 0.05. RESULTS: Total white-cell count, ANC, ALC, platelet count, and NLR were significantly higher in SCA patients than those of HbA controls. Total white-cell count, ANC, and NLR were significantly higher in the VOC than the steady-state group. PLR was not statistically different between the steady state and the VOC groups. In both groups of SCA patients, NLR was positively correlated with PLR, while percentage fetal hemoglobin was negatively correlated with percentage hemoglobin S. Hematocrit was negatively correlated with ANC and ALC in SCA patients. CONCLUSION: This study showed a significantly higher NLR and PLR in SCA than the control group. These are reflective of the roles of neutrophils, platelets, and lymphocytes in the constitutive inflammatory process. These ratios could be adopted for use in SCA patients because these markers are cheap, stable, and easily derivable from CBC.

Sickle cell anemia (SCA) pathophysiology is characterized by the activation of sickle red blood cells, reticulocytes, leukocytes, platelets, and endothelial cells, and with the expression of several inflammatory molecules. Therefore, it is conceivable that variations in levels of proinflammatory cytokines may act as a signaling of differential clinical course in SCA. Here, we evaluated the clinical impact of proinflammatory cytokines interleukin 1-β (IL-1β), interleukin 6 (IL-6), and interleukin 8 (IL-8) in 79 patients with SCA, followed in a single reference center from northeastern Brazil. The main clinical/laboratory data were obtained from patient interview and medical records. The proinflammatory markers IL-1β, IL-6, and IL-8 were evaluated by using commercially available enzyme-linked immunosorbent assay kits. According to levels of the proinflammatory markers, we observed that patients who had a higher frequency of VOC per year (P = 0.0236), acute chest syndrome (P = 0.01), leg ulcers (P = 0.0001), osteonecrosis (P = 0.0006), stroke (P = 0.0486), and priapism (P = 0.0347) had higher IL-6 levels compared with patients without these clinical complications. Furthermore, increased levels of IL-8 were found in patients who presented leg ulcers (P = 0.0184). No significant difference was found for IL-1β levels (P > 0.05). In summary, the present study emphasizes the role of inflammation in SCA pathophysiology, reveals an association of IL-8 levels and leg ulcer occurrence, and indicates that IL-6 levels can be used as a useful predictor for poor outcomes in SCA.

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.

Background and Objective: Inflammatory 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 Materials: Sixty 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

Background and objective Inflammation is implicated in the pathogenesis of most complications seen in sickle cell anemia (SCA) patients. We aimed to evaluate serum levels of two newly discovered anti-inflammatory cytokines (IL-27 and IL-37), and pro-inflammatory cytokines among Brazilian SCA patients that are not on hydroxyurea therapy (HbSS), compared with hydroxyurea-treated patients (HbSSHU) and healthy controls (HbAA). Furthermore, we demonstrated the effect of IL-27, IL-37, and heme on in vitro secretions of IL-8 in human neutrophils and monocytes. Methods A cross-sectional study of 82 consenting SCA (35 HbSS and 47 HbSSHU) patients in steady state and 49 HbAA consenting individuals. Clinical details were obtained from interviews and medical records. Serum levels of IL-27, IL-37, TGF-β, TNF-α, IL-1β, IL-6, and IL-8 were quantified by enzyme linked immunosorbent assay (ELISA). Neutrophils and monocytes were isolated from healthy controls, and cultured separately with or without cytokines (IL-27 and IL-37) and heme. Supernatant IL-8 concentration was determined by ELISA. Results Serum levels of IL-27, IL-37, IL-1β, IL-6, and IL-8 were significantly elevated in HbSS patients compared to HbAA controls. Serum IL-8 levels were significantly higher in HbSS and HbSSHU patients than in controls. IL-27 and IL-37 were positively correlated in both HbSS and HbSSHU patients. In vitro IL-8 production by IL-27 and IL-37 pre-treated neutrophils and monocytes was significantly inhibited even after heme addition. Conclusions Our findings show that IL-27 and IL-37, as well as the pro-inflammatory cytokines, are elevated in HbSS patients compared with controls, suggesting that the secretion of these anti-inflammatory cytokines is driven by the presence of pro-inflammatory cytokines. This role is probably sufficient in preventing further cellular or tissue damage but not potent enough to prevent inflammation. Therefore, IL-27 and IL-37 may be potential immuno-targets for ameliorating complications associated with elevated heme levels seen in SCA and other hemolytic anemias.

Introduction: Hypereosinophilic syndrome (HES) is a rare disorder.It is defined as eosinophilia of greater than1.5x10 9 /L persisting for at least 6 months or death before 6 months without an identifiable cause and with eosinophil-mediated organ dysfunction.We present a rare case of hypereosinophilic syndrome with severe hypokalaemia in a Nigerian female patient. Case presentation: A 43year old food vendor referred to the Haematology Department, University College Hospital, Ibadan on account of a 6-week history of cough productive of mucoid, brownish, foul smelling sputum with associated breathlessness, high grade intermittent fever,and intense pruritus. She had accompanying non-projectile,non-bloody vomiting of recently ingested meals. There was absolute eosinophilia of 83x10 9 /L and bone marrow cytology revealed marked eosinophilia with blasts of less than 5%. She also had asymptomatic severe hypokalaemia (1.9mmol/l) likely due to vomiting and reduced dietary intake.The aetiology of the hypereosinophilia could not be ascertained.She was admitted and commenced on intranasal oxygen,Tabs Loratidine, intravenous hydration.The severe hypokalaemia was corrected with IV KCL over 48hours followed with the administration of slow K tablets 600mg tds. She also had tabs Hydroxyurea for cytoreduction and Allopurinol to prevent hyperuricaemia.She improved with the above line of management. Conclusion: This appears to be the first reported case of HES with asymptomatic severe hypokalaemia in the literature. Being a rare disorder it could easily have been missed without a review of the peripheral blood film and marrow aspirate. This finding suggests a possible relationship between hypereosinophilia and hypokalemia which needs to be explored. Keywords: Hypereosinophilic syndrome, Eosinophilia, Bone marrow cytology, Hypokalemia, Hydroxyurea, Loratidine

Objective: To further highlight the fact that bone involvement is the commonest clinical manifestation of SCD both in the acute settins such painful VOC and as a source of chronic , progressive debility such as 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 aseptic necrosis of both femoral and right humeral heads, coupled with urinary tract infection((UTI) and decubitus ulcer in a young adult SCD patient at a stretch; is an unusual occurrence and deserves reporting and explanation. The determinant factors culminating in such heavy sepsis that resulted to an uninterrupted 29 weeks of hospital admission in this index patient, who had enjoyed fairly good health, deserves exploration. Various micro-organisms were sequentially cultured at various times and sites; these include E coli and Klebsiella in urine and klebsiella spp cultured in the aspirates of the affected knee joint, elbow joint and femoral osteomyelitis. Multidisciplinary approach was applied to her management and she 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 vaso-occlusive crisis (VOC) which often predisposes them to developing osteomyelitis. There is a need to have a highly organized, well-equipped and highly subsidized Sickle Cell and rehabilitation Center in order to further improve the care of SCD patients.