RESUMEN
BACKGROUND: The Immature Platelet Fraction (IPF) is an indicator of thrombopoiesis which is a useful parameter in thrombocytopenia. It demonstrates compensatory mechanisms in production of platelets, but currently not implemented in routine clinical practice. The aim of this study was to establish the reproducibility and stability of IPF, for both percentage (%-IPF) and absolute (A-IPF) measurements.Material/methods: A total of 71 samples, of which 45 for reproducibility and 26 for stability analysis, were assayed for full blood count using the Sysmex XN-10 analyser at room temperature (RT:19-25 °C). For reproducibility analysis, IPF measurements were analysed 11 times by different appraisers using the same sample, while for stability analysis, IPF was measured over fourteen hourly-intervals up to 24 h (n = 21) and then separately extended beyond the point of stability to 72 h (n = 5). RESULTS: Reproducibility analysis of %-IPF and A-IPF (n = 45) showed very reliable results, with the range of mean CV% values between 1.25-8.90% and 1.70-9.96%, respectively. On the other hand, overall, stability analysis of %-IPF and A-IPF (n = 21) at RT over 24 h showed reliable results, with pooled mean CV% values of 1.32% and 1.43%, respectively, with no significant difference between %-IPF and A-IPF (p = 0.767 and p = 0.821). All %-IPF and A-IPF values had exceeded the set acceptance criterion of stability (CV% ≥ 10.0%) before 72 h. CONCLUSIONS: Overall, %-IPF and A-IPF reproducibility and storage at RT for 24 h predominantly demonstrates the suitability of their usage for testing on the Sysmex XN-series analysers.
Asunto(s)
Plaquetas , Humanos , Reproducibilidad de los Resultados , Plaquetas/citología , Recuento de Plaquetas/instrumentación , Recuento de Plaquetas/métodos , Trombocitopenia/sangre , Trombocitopenia/diagnóstico , Trombopoyesis/fisiologíaRESUMEN
BACKGROUND: Red blood cell exchange is the cornerstone of the management for acute complications of sickle cell disease. It improves anaemia and improvesperipheral tissue oxygen delivery while at the same time reduces the proportion of circulating sickle erythrocytes. Even though automated red cell exchange is very effective in rapidly lowering the Hb S level, 24-h availability is currently not feasible for most specialist centres including our own. OBJECTIVE: Here, we describe our experience using both automated and manual red cell exchange for the management of acute sickle cell complications. METHODS: Eighty-six such episodes have been recorded between June 2011 and June 2022 comprising of 68 episodes of automated and 18 episodes of manual red cell exchange. RESULTS: The post procedure Hb S/S + C level was 18% after automated and 36% after manual red cell exchange. The platelet count dropped by 41% and 21% after automated and manual red cell exchange respectively. The clinical outcomes including need for organ support, duration of stay in the intensive care unit and overall length of hospitalisation was comparable between the two groups. CONCLUSION: In our experience, manual red cell exchange is a safe and effective alternative to an automated procedure that can be used while specialist centres are building up their capacity to offer automated red cell exchange for all patients requiring the intervention.
Asunto(s)
Anemia de Células Falciformes , Eliminación de Componentes Sanguíneos , Humanos , Transfusión de Eritrocitos/métodos , Eritrocitos , HospitalizaciónRESUMEN
BACKGROUND: Deoxygenated sickle hemoglobin (HbS) polymerization drives the pathophysiology of sickle cell disease. Therefore, direct inhibition of HbS polymerization has potential to favorably modify disease outcomes. Voxelotor is an HbS polymerization inhibitor. METHODS: In a multicenter, phase 3, double-blind, randomized, placebo-controlled trial, we compared the efficacy and safety of two dose levels of voxelotor (1500 mg and 900 mg, administered orally once daily) with placebo in persons with sickle cell disease. The primary end point was the percentage of participants who had a hemoglobin response, which was defined as an increase of more than 1.0 g per deciliter from baseline at week 24 in the intention-to-treat analysis. RESULTS: A total of 274 participants were randomly assigned in a 1:1:1 ratio to receive a once-daily oral dose of 1500 mg of voxelotor, 900 mg of voxelotor, or placebo. Most participants had sickle cell anemia (homozygous hemoglobin S or hemoglobin Sß0-thalassemia), and approximately two thirds were receiving hydroxyurea at baseline. In the intention-to-treat analysis, a significantly higher percentage of participants had a hemoglobin response in the 1500-mg voxelotor group (51%; 95% confidence interval [CI], 41 to 61) than in the placebo group (7%; 95% CI, 1 to 12). Anemia worsened between baseline and week 24 in fewer participants in each voxelotor dose group than in those receiving placebo. At week 24, the 1500-mg voxelotor group had significantly greater reductions from baseline in the indirect bilirubin level and percentage of reticulocytes than the placebo group. The percentage of participants with an adverse event that occurred or worsened during the treatment period was similar across the trial groups. Adverse events of at least grade 3 occurred in 26% of the participants in the 1500-mg voxelotor group, 23% in the 900-mg voxelotor group, and 26% in the placebo group. Most adverse events were not related to the trial drug or placebo, as determined by the investigators. CONCLUSIONS: In this phase 3 randomized, placebo-controlled trial involving participants with sickle cell disease, voxelotor significantly increased hemoglobin levels and reduced markers of hemolysis. These findings are consistent with inhibition of HbS polymerization and indicate a disease-modifying potential. (Funded by Global Blood Therapeutics; HOPE ClinicalTrials.gov number, NCT03036813.).
Asunto(s)
Anemia de Células Falciformes/tratamiento farmacológico , Antidrepanocíticos/administración & dosificación , Benzaldehídos/administración & dosificación , Hemoglobina Falciforme/efectos de los fármacos , Hemoglobinas/metabolismo , Pirazinas/administración & dosificación , Pirazoles/administración & dosificación , Adolescente , Adulto , Anemia de Células Falciformes/sangre , Antidrepanocíticos/efectos adversos , Antidrepanocíticos/uso terapéutico , Benzaldehídos/efectos adversos , Biomarcadores/sangre , Niño , Relación Dosis-Respuesta a Droga , Método Doble Ciego , Quimioterapia Combinada , Femenino , Hemoglobina Falciforme/metabolismo , Hemólisis/efectos de los fármacos , Humanos , Hidroxiurea/uso terapéutico , Análisis de Intención de Tratar , Masculino , Persona de Mediana Edad , Polimerizacion/efectos de los fármacos , Pirazinas/efectos adversos , Pirazoles/efectos adversos , Adulto JovenRESUMEN
BACKGROUND: Posttransfusion hyperhemolysis syndrome is a rare but life-threatening form of delayed hemolytic transfusion reaction with lysis of both transfused and autologous red cells, seen predominantly in patients with sickle cell disease. Macrophage activation is thought to play a major role in its pathophysiology. Standard treatment is with intravenous immunoglobulin and steroids but refractory cases pose a major clinical problem. Tocilizumab is a humanized monoclonal antibody against the IL-6 receptor that can inhibit IL-6 induced macrophage activation. METHODS AND MATERIALS: We describe the case of a 33-year-old woman with sickle cell anemia and posttransfusion hyper hemolysis syndrome refractory to standard therapy, treated with Tocilizumab. We also review all cases reported in the literature where Tocilizumab was used for posttransfusion hyperhemolysis. RESULTS: Treatment with Tocilizumab was well tolerated with no observed adverse events. There was no further drop in Hb after day 2 of treatment with subsequent continuous gradual improvement. Her bilirubin dropped significantly after the first dose and continued to improve, while ferritin and LDH reduced significantly after day 2 of treatment with Tocilizumab and continued to drop thereafter. Like in our case, all other cases in the literature where Tocilizumab was used for posttransfusion hyperhemolysis led to rapid clinical responses and no adverse events. DISCUSSION: Even though the number of cases of posttransfusion hyper hemolysis syndrome treated with Tocilizumab are few, they have all been associated with rapid clinical responses with no observed adverse events suggesting that the role of Tocilizumab in this context needs to be further explored.
Asunto(s)
Anemia de Células Falciformes , Hemólisis , Adulto , Anemia de Células Falciformes/complicaciones , Anemia de Células Falciformes/terapia , Anticuerpos Monoclonales Humanizados/uso terapéutico , Femenino , Humanos , Inmunoglobulinas Intravenosas , SíndromeRESUMEN
Sickle cell disease results in systemic inflammation even at steady state and this is accentuated during acute crises. The plasma of affected patients contains several proinflammatory cytokines as well as adhesion molecules and prothrombotic factors. This environment promotes further red cell sickling while many of these substances can cause direct tissue toxicity and end-organ damage. Even though red cell transfusion, whether simple or exchange, is the mainstay of treatment of severe acute complications, addition of therapeutic plasma exchange could potentially provide additional benefit by removing such harmful substances. Here, we describe two cases where therapeutic plasma exchange was used. The first involved a patient with the acute chest syndrome who despite adequate red cell exchange remained significantly hypoxic and in severe pain. We therefore proceeded to perform plasma exchange; this led to rapid clinical improvement and resolution of his symptoms. The second case involved a patient with intractable chest wall pain and impending acute chest syndrome; this patient also had a past history of hyperhaemolysis. The patient underwent therapeutic plasma exchange with very rapid resolution of the pain, avoidance of any respiratory deterioration and full recovery. We also give a brief summary of our previous experience using plasma exchange in patients with sickle cell disease. Plasma exchange was well tolerated with no adverse events in all cases we have treated, led to rapid resolution of pain irrespective of primary indication and in the majority of cases to a favourable clinical outcome.
Asunto(s)
Síndrome Torácico Agudo , Anemia de Células Falciformes , Síndrome Torácico Agudo/complicaciones , Síndrome Torácico Agudo/terapia , Anemia de Células Falciformes/complicaciones , Anemia de Células Falciformes/terapia , Transfusión de Eritrocitos/efectos adversos , Transfusión de Eritrocitos/métodos , Humanos , Dolor , Intercambio Plasmático/efectos adversosRESUMEN
Sickle cell disease and thalassaemia are life-long haematological diseases that can impact the quality of life of patients. This impact on quality of life can require intermittent psychological input throughout the lifespan for management. Managing everyday life during the COVID-19 pandemic could be challenging for people with these health conditions, which could impact their health, their mood and anxiety, their perception of control, and their engagement with their regular healthcare services. This report describes the characteristics of these health conditions and discusses reflections, from a specialist psychology service working with this clinical population, about the impact of COVID-19 on patient engagement with the service. The main aim of this report is to highlight the relevance and usefulness of videoconferencing as a therapy format, suggest implications for further service development and suggest alternate ways of working therapeutically with clients.
Asunto(s)
Anemia de Células Falciformes , COVID-19 , Talasemia , Anemia de Células Falciformes/complicaciones , Anemia de Células Falciformes/terapia , Humanos , Pandemias , Calidad de Vida , Talasemia/complicaciones , Talasemia/terapiaRESUMEN
Fat embolism syndrome in sickle cell disease is associated with great mortality, while more than half of survivors suffer severe neurological sequelae. Release of fat droplets leads to obstruction of the microcirculation as well as generation of proinflammatory cytokines that can cause direct tissue injury. Red cell exchange transfusion can be life-saving but the addition of therapeutic plasma exchange may further improve outcomes by removing such inflammatory mediators. Here, we describe the case of a 27-year-old male patient with sickle cell anaemia presenting with typical features of fat embolism syndrome including neurological involvement with greatly reduced level of consciousness. MRI of his brain showed multiple widespread microhemorrhages giving the characteristic "star field" pattern but also a cytotoxic lesion of the corpus callosum, known to be the result of direct neurotoxicity by proinflammatory cytokines. The patient underwent emergency red cell exchange transfusion leading only to modest clinical improvement but fully regained consciousness after three cycles of therapeutic plasma exchange. This case highlights the deleterious effect of the hyperinflammatory state characteristic of many sickle cell complications and supports further exploring the potential benefit from plasma exchange as an adjunct to red cell exchange in order to remove proinflammatory cytokines during acute complications of sickle cell disease.
Asunto(s)
Anemia de Células Falciformes/complicaciones , Embolia Grasa/etiología , Recambio Total de Sangre/efectos adversos , Intercambio Plasmático/efectos adversos , Adulto , Embolia Grasa/mortalidad , Embolia Grasa/fisiopatología , Humanos , Masculino , Análisis de SupervivenciaRESUMEN
Voxelotor is a novel agent in the management of sickle cell disease. It is an inhibitor of Hb S (HBB: c.20A>T) polymerization that reversibly binds to hemoglobin (Hb), stabilizing it in the oxygenated state that has been shown to reduce hemolysis and to improve anemia. Four patients in our institution are receiving treatment with Voxelotor as part of clinical studies. All four showed a characteristic change in the appearance of Hb S by high performance liquid chromatography (HPLC) soon after commencing treatment. A second peak was identified eluting at the Hb D window. Cellulose acetate membrane and agar gel electrophoresis only identified a band at the Hb S position. The patients' Hb level or clinical conditions were not adversely affected. Our findings indicate that patients receiving Voxelotor invariably display this unique pattern by HPLC. As there will be an increasing number of patients treated with this agent, it is important to be aware of this characteristic HPLC appearance for diagnostic and treatment monitoring purposes.
Asunto(s)
Anemia de Células Falciformes , Hemoglobina Falciforme , Anemia de Células Falciformes/diagnóstico , Anemia de Células Falciformes/tratamiento farmacológico , Benzaldehídos/efectos adversos , Cromatografía Líquida de Alta Presión , Hemoglobina Falciforme/análisis , Humanos , Pirazinas , PirazolesRESUMEN
BACKGROUND: 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 of life (HRQoL) in the US sickle cell disease (SCD) population. The study objective was to test the validity and reliability of the ASCQ-Me for use in the UK. METHODS: The US ASCQ-Me, Hospital Anxiety and Depression Scale (HADS), self-reported symptoms, and Medical Outcome Survey Short Form 36 (SF-36) were administered to 173 patients with SCD. Clinical severity was assessed by the number of painful episodes indicated by hospital admissions. RESULTS: The results showed that the item banks of the UK ASCQ-Me had good internal consistency. Anxiety and depression were strongly correlated with the emotional, and social item banks of the UK ASCQ-Me, with moderate correlations between the UK ASCQ-Me item banks and SF-36 components suggesting convergent validity. A confirmatory factor analysis confirmed the conceptual framework of the scale as being the same as the US ASCQ-Me, indicating construct validity. Known groups validity was found, with the ASCQ-Me being able to differentiate by SCD severity groups. CONCLUSION: The analysis of the sample shows evidence of both validity and reliability of the ASCQ-Me for use in the UK SCD population.
Asunto(s)
Anemia de Células Falciformes/psicología , Medición de Resultados Informados por el Paciente , Calidad de Vida , Adulto , Ansiedad/psicología , Depresión/psicología , Análisis Factorial , Femenino , Humanos , Masculino , Persona de Mediana Edad , Psicometría , Reproducibilidad de los Resultados , Índice de Severidad de la Enfermedad , Reino UnidoRESUMEN
Delayed hemolytic transfusion reaction (DHTR) is a life-threatening complication in patients with sickle cell disease, characterized by difficulties in diagnosis and management. Certain reports have suggested successful salvage treatment with the terminal complement inhibitor, eculizumab. We here report evidence of complement activation and successful complement inhibition with one dose of eculizumab in an adult sickle cell disease patient presenting DHTR with hyperhemolysis. A 21-year old female sickle cell disease patient [Hb S (HBB: c.20A>T)/ß-thalassemia (ß-thal)] presented at our Adult Thalassaemia Unit, Hippokration General Hospital of Thessaloniki, Thessaloniki, Greece, with headaches, perioral numbness and pain in both antibrachia. The patient was admitted with the diagnosis of vaso-occlussive crisis (VOC) and treated symptomatically. On her third day of admission, due to hemoglobin (Hb) value of 6.9 g/dL with increase in reticulocytes, the patient was transfused with one unit of compatible leukodepleted red blood cells (RBC). The following day, hemolytic parameters increased, although Coombs, panel antibodies and screening tests were negative. Five days later, she again received a unit of RBCs, resulting in another increase of hemolytic parameters. During the following 2 days, there was a dramatic decrease of Hb levels to 5.4 g/dL with reticulocytes at 6.0%, negative Coombs testing and negative alloantibodies. Based on these findings, we recognized the syndrome of DHTR with hyperhemolysis. Given the lack of immediate access to other treatment, we administered intravenous rituximab, immunoglobulins and corticosteroids. As there was no response, one dose of eculizumab (900 mg) was then administered with ciprofloxacin as prophylaxis. The patient remains well 6 months post treatment.
Asunto(s)
Anemia de Células Falciformes/complicaciones , Anticuerpos Monoclonales Humanizados/administración & dosificación , Reacción a la Transfusión/etiología , Anemia de Células Falciformes/terapia , Profilaxis Antibiótica , Anticuerpos Monoclonales Humanizados/uso terapéutico , Ciprofloxacina , Femenino , Hemólisis , Humanos , Terapia Recuperativa , Resultado del Tratamiento , Adulto JovenRESUMEN
Two main sub-phenotypes have been described in sickle cell disease: one with higher baseline haemoglobin and a higher rate of painful crises and one with lower baseline haemoglobin, increased markers of haemolysis and a higher incidence of pulmonary hypertension, priapism and leg ulcers. We compared the patterns of response to regular automated red cell exchange transfusion over a five-year period of 21 patients with recurrent painful crises from the first group and 3 patients with pulmonary hypertension and 5 with recurrent severe stuttering priapism form the second and found them to be distinctly different. Response for pain is slow and increases gradually over years. The most pronounced clinical benefit and the one that appears first is a reduction in the severity rather than the frequency of painful crises. In contrast to the slow and gradual response we see for pain, response of patients with pulmonary hypertension and priapism is immediate with significant clinical improvement even after the first transfusion. The response appears to be directly correlated to the HbS level as the symptoms of both conditions invariably recur rapidly when transfusions are delayed or discontinued but resolve again once they are re-instituted.
Asunto(s)
Anemia de Células Falciformes/complicaciones , Transfusión Sanguínea/métodos , Adulto , Anemia de Células Falciformes/terapia , Femenino , Humanos , Masculino , Persona de Mediana EdadRESUMEN
The painful vaso-occlusive crisis is the most common acute manifestation of sickle cell disease resulting in poor quality of life and high utilisation of hospital facilities. The main disease modifying strategy is treatment with hydroxycarbamide. For patients intolerant or who fail hydroxycarbamide, chronic transfusions are an alternative. Automated red cell exchange transfusion (ARCET) are more effective in lowering rapidly the HbS level while avoiding iron overload. As they require specialised equipment and specially trained staff while utilising higher volumes of blood, there have been concerns regarding the costs involved. We retrospectively analysed data on 23 patients who have been on a regular programme for 1-5 years and found that their utilisation of hospital services reduced by 20%, 48%, 58%, 71%, and 79% after 1, 2, 3, 4 and 5 years respectively. The overall mean annual cost of care per patient was £9702 and £2378 higher than baseline after the 1st and 2nd years of ARCET respectively and then reduced by £5486, £8317, and £14,664 after the 3rd, 4th and 5th year of ARCET respectively indicating that ARCET leads to cost savings to health services in the medium to long term due to reduction in hospital attendance of these patients.
Asunto(s)
Anemia de Células Falciformes/terapia , Costos y Análisis de Costo/tendencias , Transfusión de Eritrocitos/métodos , Recambio Total de Sangre/métodos , Dolor/tratamiento farmacológico , Adulto , Femenino , Humanos , Masculino , Persona de Mediana Edad , Calidad de Vida , Estudios Retrospectivos , Factores de Tiempo , Adulto JovenRESUMEN
BACKGROUND: Immature platelet fraction (IPF) estimation is a non-invasive and sensitive test that is available on recently introduced Sysmex XN-series of automated haematology analysers. It is a direct cellular indicator of thrombopoiesis. The aim of this study was to establish reference intervals for IPF, for both absolute (A-IPF) and percentage (%-IPF) measurements. MATERIAL AND METHODS: A total of 2366 samples that met the inclusion criteria were assayed for full blood count on the Sysmex XN-10 and a non-parametric percentile method was used for calculating the reference intervals. RESULTS: After the outliers were excluded, the reference interval for %-IPF and A-IPF on Sysmex XN-10 were 1.6-10.1% and 4.37-23.21 × 109/L in total individuals, respectively. There was a statistical significance noted between the sexes (p = .004) for %-IPF, therefore a sex-specific reference interval was established, which was 1.8-10.0% for the males and 1.5-10.1% for females. No significant difference in sex status for A-IPF and age status for both %-IPF and A-IPF was observed. A very poor correlation was estimated between age versus %-IPF, ρ = 0.0156, and age versus A-IPF, ρ = -0.0023, indicating that there is no overall biological relationship between age and these parameters. As expected, a strong correlation between %-IPF and A-IPF was noted which could be attributed to their inter-relatedness. CONCLUSIONS: This large-scale study showed comparable reference intervals with the previous studies for %-IPF and A-IPF in a UK population. It found the need to establish sex-specific reference intervals for %-IPF, but not for A-IPF, whereas reference intervals were found to be stable across the age range.
Asunto(s)
Recuento de Plaquetas/instrumentación , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Plaquetas , Femenino , Humanos , Masculino , Persona de Mediana Edad , Valores de Referencia , Reino Unido , Adulto JovenAsunto(s)
Anemia de Células Falciformes/inmunología , Vacunas contra la COVID-19/administración & dosificación , COVID-19/complicaciones , Encuestas y Cuestionarios/estadística & datos numéricos , Adulto , Anemia de Células Falciformes/epidemiología , Anemia de Células Falciformes/mortalidad , Anemia de Células Falciformes/virología , COVID-19/diagnóstico , COVID-19/epidemiología , COVID-19/inmunología , Vacunas contra la COVID-19/efectos adversos , Vacunas contra la COVID-19/inmunología , Comorbilidad , Femenino , Genotipo , Hospitalización/estadística & datos numéricos , Hospitalización/tendencias , Humanos , Incidencia , Londres/epidemiología , Masculino , Recuperación de la Función , SARS-CoV-2/genéticaRESUMEN
We report here our experience with regular automated red cell exchange transfusion for the management of chronic complications of sickle cell disease in 50 patients in our institution from June 2011 to December 2014. The mean sickle hemoglobin level was 44% and 8.5% pre- and post-transfusion, respectively. Platelets were reduced by a mean 70% during the procedure with a count of less than 50 × 109 /l in 6% of cases. The alloimmunization rate was 0.065/100 units of red cells with no hemolytic reactions. Patients with no iron overload at baseline showed no evidence of iron accumulation with a mean liver iron concentration of 1.6 mg/g dry tissue and 1.9 mg/g dry tissue at baseline and 36 months, respectively. All six patients with pre-existing iron overload and on chelation therapy, showed a gradual reduction of their liver iron concentration and two patients could discontinue chelation during the follow-up period. Seventy percentage of patients who were on the programme for recurrent painful crises showed a sustained reduction in the number of emergency hospital attendances; the mean number of days in hospital for emergency treatment was 103 in the year prior to commencing ARCET and reduced to 62 (40%) after the first 12 months, 51 (50%) after 24 months, and 35 days (66%) after 36 months. J. Clin. Apheresis 31:545-550, 2016. © 2015 Wiley Periodicals, Inc.
Asunto(s)
Anemia de Células Falciformes/terapia , Transfusión de Eritrocitos/normas , Automatización , Manejo de la Enfermedad , Transfusión de Eritrocitos/métodos , Humanos , Hierro/metabolismo , Tiempo de Internación , Dolor , Seguridad del Paciente , Recuento de Plaquetas , Resultado del TratamientoAsunto(s)
Anemia de Células Falciformes/complicaciones , Infecciones por Coronavirus/complicaciones , Neumonía Viral/complicaciones , Adulto , Betacoronavirus , COVID-19 , Infecciones por Coronavirus/diagnóstico , Infecciones por Coronavirus/terapia , Femenino , Humanos , Masculino , Persona de Mediana Edad , Pandemias , Neumonía Viral/diagnóstico , Neumonía Viral/terapia , SARS-CoV-2 , Reino Unido , Adulto JovenAsunto(s)
Anemia de Células Falciformes/terapia , Anticuerpos Monoclonales Humanizados/administración & dosificación , Transfusión de Eritrocitos , Hemólisis/efectos de los fármacos , Reacción a la Transfusión/tratamiento farmacológico , Adulto , Anemia de Células Falciformes/sangre , Humanos , Masculino , Síndrome , Reacción a la Transfusión/sangreRESUMEN
AIMS: Transfused patients with sickle cell disease (SCD) are at risk of iron overload and identifying such patients is important to prevent associated complications. Our aim was to assess the efficacy of serial serum ferritin (SF) measurements in identifying patients with hepatic iron overload as assessed by liver MRI and its usefulness in guiding decision making regarding chelation therapy. PATIENTS/METHODS: We retrospectively compared the results of 49 liver MRI scans (LS) with the median serum ferritin (MSF) values for 28 patients in our institution. RESULTS: We found a nonlinear increment of MSF with increasing liver iron concentration (LIC). 18.4% and 47.4% of abnormal LSs corresponded to MSF <1000 mcg/L and <2000 mcg/L, respectively. 30.4% of patients with LIC of 7 mg/g dry weight or higher had a MSF <2000 mcg/L. In 38.5% of patients receiving chelation, MSF offered little information regarding the efficacy of treatment and was sometimes misleading. CONCLUSION: Serial serum ferritin measurements in adult transfused patients with sickle cell disease have a low sensitivity for identifying patients with iron overload and are of limited value in guiding decision making regarding initiation or monitoring of chelation therapy. The iron status of such high risk patients should be assessed by more definitive ways such as MRI.