A5049353540- Hemoglobinopathies and Related Disorders
- Blood groups and transfusion
- Iron Metabolism and Disorders
- Erythrocyte Function and Pathophysiology
- Parvovirus B19 Infection Studies
- Bone and Joint Diseases
- Chronic Lymphocytic Leukemia Research
- Platelet Disorders and Treatments
- Fungal Infections and Studies
- Lymphoma Diagnosis and Treatment
- Autopsy Techniques and Outcomes
- Blood properties and coagulation
- Antifungal resistance and susceptibility
- Autoimmune Bullous Skin Diseases
- Neurological and metabolic disorders
- Hematological disorders and diagnostics
- Palliative Care and End-of-Life Issues
- Infectious Encephalopathies and Encephalitis
- Immune Cell Function and Interaction
- Clinical Laboratory Practices and Quality Control
- Acute Myeloid Leukemia Research
- Complement system in diseases
- Genital Health and Disease
- Dermatological and COVID-19 studies
- Neonatal Health and Biochemistry
Homerton University Hospital NHS Foundation Trust
2015-2024
National Health Service
2011-2021
Alexandria University
2019
St Bartholomew's Hospital
2007-2013
Barts Health NHS Trust
2008-2010
Royal London Hospital
2007
Deoxygenated sickle hemoglobin (HbS) polymerization drives the pathophysiology of cell disease. Therefore, direct inhibition HbS has potential to favorably modify disease outcomes. Voxelotor is an inhibitor.
Fat embolism syndrome is a devastating complication of sickle cell disease resulting from extensive bone marrow necrosis and associated with high mortality rates, while survivors often suffer severe neurological sequelae. Despite that, the remains under-recognised under-diagnosed. Paradoxically, it affects exclusively patients mild forms disease, predominantly HbSC HbSβ+. A significant number cases occur in context human parvovirus B19 infection. We provide here brief summary existing...
Hospital autopsy rates have been falling steadily over recent decades. One factor that has implicated in this decline is the perception general public views postmortem examinations unfavourably and often makes clinicians reluctant to discuss with families seek their consent. The aim of study was test assumption.In division lymphoid malignancies at St Bartholomew's Hospital, we suggested discussed it depth all patients who died hospital an 8-month period order assess whether rate could be...
Diagnosis of invasive aspergillosis (IA) remains a challenge as the clinical manifestations are not specific, and histological diagnosis is often unfeasible. The 2002 European Organization for Research Treatment Cancer (EORTC) National Institute Allergy Infectious Diseases Mycoses Study Group (MSG) criteria classification cases into possible, probable or proven were revised in 2008. Our objective was to analyze impact these revisions on IA. A retrospective analysis 589 high risk...
Delayed hemolytic transfusion reaction (DHTR) is a life-threatening complication in patients with sickle cell disease, characterized by difficulties diagnosis and management. Certain reports have suggested successful salvage treatment the terminal complement inhibitor, eculizumab. We here report evidence of activation inhibition one dose eculizumab an adult disease patient presenting DHTR hyperhemolysis. A 21-year old female [Hb S (HBB: c.20A>T)/β-thalassemia (β-thal)] presented at our Adult...
Red cell transfusion represents one of the cornerstones chronic management sickle disease, as well its acute complications. Automated red exchange can rapidly lower number circulating erythrocytes, without causing iron overload. Here, we describe our experience, having offered this intervention since 2011. A transient reduction in platelet count by 61% was observed after procedure. This not associated with any haemorrhagic Despite exposure to large volumes blood, alloimmunisation rate only...
We report here our experience with regular automated red cell exchange transfusion for the management of chronic complications sickle disease in 50 patients institution from June 2011 to December 2014. The mean hemoglobin level was 44% and 8.5% pre- post-transfusion, respectively. Platelets were reduced by a 70% during procedure count less than × 109 /l 6% cases. alloimmunization rate 0.065/100 units cells no hemolytic reactions. Patients iron overload at baseline showed evidence...
Abstract Background Posttransfusion hyperhemolysis syndrome is a rare but life‐threatening form of delayed hemolytic transfusion reaction with lysis both transfused and autologous red cells, seen predominantly in patients sickle cell disease. Macrophage activation thought to play major role its pathophysiology. Standard treatment intravenous immunoglobulin steroids refractory cases pose clinical problem. Tocilizumab humanized monoclonal antibody against the IL‐6 receptor that can inhibit...
Immature platelet fraction (IPF) estimation is a non-invasive and sensitive test that available on recently introduced Sysmex XN-series of automated haematology analysers. It direct cellular indicator thrombopoiesis. The aim this study was to establish reference intervals for IPF, both absolute (A-IPF) percentage (%-IPF) measurements.A total 2366 samples met the inclusion criteria were assayed full blood count XN-10 non-parametric percentile method used calculating intervals.After outliers...
Fat embolism syndrome (FES) is a rare complication of sickle-cell disease (SCD) associated with extremely high mortality rates. It affects predominantly non-SS patients and those previously mild disease. Rapid institution exchange transfusion an aim to reduce HbS very low levels as soon FES suspected can be life-saving.
Abstract Aims Transfused patients with sickle cell disease ( SCD ) are at risk of iron overload and identifying such is important to prevent associated complications. Our aim was assess the efficacy serial serum ferritin SF measurements in hepatic as assessed by liver MRI its usefulness guiding decision making regarding chelation therapy. Patients/Methods We retrospectively compared results 49 scans LS median MSF values for 28 our institution. Results found a nonlinear increment increasing...
Galactomannan (GM) is widely used for detection of invasive aspergillosis in high-risk haemato-oncology patients. Recent publications have reported a lack repeatability GM detection. The objective this retrospective study was to assess the levels during storage clinical samples. In screening strategy, positive sera were repeat tested as per manufacturer’s recommendations. Short-term (ST) samples at +4 °C while long-term (LT) −80 °C. Bronchoalveolar (BAL) fluid also repeating after ST and LT...
This study aimed to define the sex-divided reference intervals for platelet indices (mean volume (MPV), distribution width (PDW), large cell ratio (P-LCR) and plateletcrit (PCT)) on Sysmex XN-10.A total of 2376 samples were assayed full blood count XN-10 haematology analyzer. After removing outliers, calculated using mean±2SD. The P value 0.05 was adopted denote statistical significance.There a significance (α=0.05) between MPV (p=0.007), P-LCR (p=0.015) PCT (p<0.001), thus separate these...
The Adult Sickle Cell Quality of Life Measurement Information System (ASCQ-Me) has been shown to be a reliable and valid questionnaire measuring health-related quality life (HRQoL) in the US sickle cell disease (SCD) population. study objective was test validity reliability ASCQ-Me for use UK. ASCQ-Me, Hospital Anxiety Depression Scale (HADS), self-reported symptoms, Medical Outcome Survey Short Form 36 (SF-36) were administered 173 patients with SCD. Clinical severity assessed by number...
Abstract Fat embolism syndrome (FES) is a rare life‐threatening condition that particularly seen in milder forms of sickle cell disease (SCD). Widespread systemic fat emboli are generated the context extensive bone marrow necrosis. Multi‐organ failure with high morbidity and mortality may quickly develop. Infection Parvovirus B19 common precipitant. Here, authors report case 35‐year‐old Afro‐Caribbean man HbSC who presented FES having tested positive for SARS‐COV‐2. He rapidly became...