Objectives The aim of this study was to assess the impact of COVID-19 on the morbidity and mortality on vasooclusive crisis (VOC) in sickle cell anaemia (SCA) patients. Methods One hundred patients with(fifty) or without COVID-19 PCR positivity(fifty), were enrolled in a prospective cohort study after signing a written informed consent. Results The COVID-19 positive patients had significantly higher median VOC episodes/year i.e 3 /year (IQR, 1-6 /year) v/s 2 /year (IQR, 2-12 /year) (p < 0.05) respectively, however the need for hospitalization was similar. There was a higher culture proven infection in COVID-19 negative group (P = 0.05). COVID-19 positive group had more osteonecrosis (p < 0.05), splenic sequestration, splenomegaly, and hepatic crisis (P values 0.05, 0.006 and 0.02 respectively). Symptoms of fever, cough, fatigue, abdominal pain and anosmia were significantly higher (p < 0.05) in the COVID-19 positive patients. Both cohorts, showed a fall in the mean haemoglobin, lymphocyte subset, platelets, and reticulocytes, whereas, the LDH and ferritin were significantly elevated. In SCA COVID-19 positive patients the rise in WBC, reticulocyte%, platelets, and ferritin was subdued (p < 0.05). Two died in COVID-19 positive, whereas three died in the COVID-19 negative, without statistically significant difference in mortality. Conclusions Although COVID-19 infection may have triggered the onset of VOC, it did not significantly influence the morbidity or mortality in this SCA cohort.

Vaccines against acute respiratory syndrome Coronavirus 2(SARS-CoV2) are critical weapons to control the spread of the deadly Coronavirus 2019(COVId-19) virus worldwide. Although these vaccines are generally safe, their widespread use has produced reports of rare complications, including vaccine-induced immune thrombotic thrombocytopenia (VIITT), particularly in connection with ChAdOx1 nCov-19. We have identified three cases of sickle cell disease (SCD) experiencing a severe vaso-occlusive crisis (VOC) shortly after the vaccine. Despite being stable for a long time, they had fever with tachycardia, along with a significant rise in WBC, liver enzymes, particularly alkaline phosphate, with a remarkable drop in hemoglobin, and platelets and one of them probably had fatal TTP like syndrome. Given these findings, physicians and patients should exercise caution when taking this type of vaccine and be aware of these safety concerns.

Objectives Many barriers contribute to the underutilization of hydroxyurea (HU) in the treatment of sickle cell disease (SCD), and adherence to its use is often reported to be suboptimal. It is important to have information on the safety of HU in patients with SCD. Our study assessed the pattern of use, patients’ adherence to medication, discontinuation of use, and safety of HU in patients with SCD. Methods This cross-sectional study was conducted in the department of medicine of a referral hospital in Oman over five months and included a review of patient files and patient interview. Approval was obtained from the Regional Research and Ethics Committee of the A'Dakhiliyah Governorate and the hospital administration. The parameters were compared between groups using the chi-square test. Results Of 298 patients studied, 128 (43.0%) had used HU at some points. The difference in the prevalence of HU use was statistically significant based only on age ( p = 0.014), with younger patients more likely to be currently using HU or used HU in the past. The majority of patients were adherent (82.5%) based on self-reported adherence. The prevalence of discontinuation (temporary or permanent) of HU use was high (57.0%), and suspected adverse drug reaction (ADR) was the most common reason. Among those who had never used HU, 33.7% of patients had an indication for the initiation of HU. A quarter of patients who used HU developed a suspected ADR, with blood abnormalities being the commonest. The duration of HU use influenced ADR prevalence ( p = 0.015). Conclusions Among the current users of HU, the majority of the patients were adherent based on self-reported medication adherence. The prevalence of discontinuation of HU use and instances of non-initiation of HU among those indicated were high. A larger study, ideally of a prospective nature, in various governorates of Oman, would provide a wider picture at the national level.

Sickle cell disease (SCD) is a major public health burden worldwide with increasing morbidity and mortality. We studied the risk factors associated with mortality in SCD patients, between the years 2006 and 2020 at three hospitals in Oman. We analyzed clinical manifestations, haematological, biochemical, and radiological parameters, use of antibiotics, and blood and exchange transfusions. Our cohort included 123 patients (82 males, 41 females), with a median age of 27 (IQ 21–35 years). SCD related complications included acute chest syndrome (ACS) in 52.8%, splenic sequestration in 21.1%, right upper quadrant syndrome in 19.5%, more than > 6 VOC /year in 17.9%, and stroke in 13.8%. At the terminal event, patients had cough, reduced O2 saturation, crepitation and fever in 24.4%, 49.6%, 53.6% and 68.3% respectively. Abnormal chest x-ray and chest CT scan were seen in 57.7%, and 76.4% respectively. Laboratory parameters showed a significant drop in hemoglobin (Hb) and platelet counts from baseline, with a significant rise in WBC, LDH and CRP from baseline (p < 0.05, Wilcoxon Signed Ranks test).. All patients received antibiotics, whereas, 95.9% & 93.5% received simple blood transfusions, and exchange transfusions respectively, and 66.6% required non-invasive ventilation. Among the causes of death, ACS is seen in 32 (26%), sepsis in 49 (40%), and miscellaneous in 42 (34%). Sudden death was seen in 32 (26%) of patients. Male gender, with low HbF, rapid drop in Hb and platelet, and increased in WBC, LDH, ferritin, and CRP, correlated significantly with mortality in this cohort.

Hemoglobin La Desirade (Hb La Desirade) is an unstable hemoglobin variant characterized by amino acid Alanine (Ala) replacing Valine (Val) at position 129 (H7) in the beta chain. Hb La Desirade exhibits a decreased oxygen affinity and normal heme-heme interaction. Interestingly, on analysis by standard electrophoresis, it migrates in the same region as normal HbA, and HbA actually represents a combination of HbA and Hb La Desirade together. This variant was reported as compound heterozygous with other Hemoglobin variants such as HbS, HbC or beta thalassemia, and more recently with Southeast Asian ovalocytosis and Hb Louisville with varying clinical manifestations.  Herein, we describe the clinical and laboratory findings in a number of Omani Arab families who presented to our service for various reasons, presenting with Hemoglobin La Desirade with sickle gene and alpha thalassemia. Our patients with Hb La Desirade trait, were clinically asymptomatic with no evidence of anemia. However when it is associated with other abnormal hemoglobin variants such as HbS, leading to sickle/La Desirade compound heterozygosity, there was mild anemia with significant degree of hypochromia and microcytosis. The most striking feature was that the levels of HbS and HbA were almost equal on HPLC, and these cases could be misdiagnosed as sickle cell trait (SCT). However, the levels of Hb S in these compound heterozygotes (40.4-45.9) were higher than normally seen for the diagnosis of SCT in this population.