Iron overload is a determinant of morbidity and mortality in adult patients with sickle cell disease

2001 ◽  
Vol 38 (1, Suppl 1) ◽  
pp. 30-36 ◽  
Author(s):  
Samir K. Ballas
Keyword(s):  
Sickle Cell Disease ◽  
Iron Overload ◽  
Sickle Cell ◽  
Adult Patients ◽  
Morbidity And Mortality ◽  
Cell Disease

Transfusional Iron Overload In An Adult Sickle Cell Disease Population: Epidemiology, Prevalence, and Management

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 1005-1005 ◽  
Author(s):  
James Son ◽  
Hongyan Xu ◽  
Nadine J Barrett ◽  
Leigh G Wells ◽  
Latanya Bowman ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Iron Overload ◽  
Sickle Cell ◽  
Serum Ferritin ◽  
Chelation Therapy ◽  
Organ Damage ◽  
Morbidity And Mortality ◽  
Genotype Distribution ◽  
Cell Disease ◽  
The Mean

Abstract Transfusional iron (Fe) overload remains a significant problem among patients with chronic, transfusion dependent anemias, especially in transfusion dependent ß-thalassemia (Thal) syndromes. If not treated vigorously with chelation, Fe overload in Thal is associated with significant organ damage, especially with chronic liver disease and cardiac abnormalities which can contribute to morbidity and mortality. In recent decades, the significance of Fe overload in sickle cell disease (SCD) has also been recognized especially among pediatric patients on chronic transfusion regimens predominantly for primary and secondary prevention of stroke. The prevalence and significance of this problem among adult SCD patients is less clear, although it is widely believed that episodic, mostly unnecessary transfusion practices play a more prominent role in this patient population. There have been reports of an association between iron overload and increased morbidity and mortality among adult SCD patients; it has also been speculated that the chronic inflammatory state that exists in SCD affords some degree of protection against severe organ damage through upregulation of hepcidin and sequestration of Fe in these patients. We performed a retrospective review of 635 adult SCD patients followed at our Center to define and ascertain the epidemiology, prevalence, etiology, and clinical correlates of transfusional Fe overload. Fe overload was defined as two consecutive serum ferritin values of > 1000 ng/ml. 80 patients (12.6%) met this criterion. Of these, 38 were male and 42 were female. Genotype distribution was: 73 SS, 3 S-β+ thal, 2 S-β0 thal and 2 SC. The mean age was 35.9 (range 18-69). Out of the 80 patients with transfusional Fe overload, 24 (30%) were/had been on a chronic transfusion regimen (23 for secondary or primary stroke prevention and one for childhood cardiomyopathy). Seventy percent of the patients (n=56) developed Fe overload from episodic transfusions predominantly performed at outlying community hospitals. The mean highest ferritin value was 4991 ng/ml (range 1,052-16,500). There was no correlation between ferritin levels and the number of hospitalizations or painful episodes (p=0.9). Thirty seven patients (46.2%) had a history of chelation therapy (with desferoxamine, deferasirox, or both). In 25 patients who have been on deferasirox for a period of 6 months or more, serum ferritin levels decreased from 4452.3 to 3876.6 ng/ml (p=0.3239). Our retrospective study shows that transfusional Fe overload is not rare among adults with SCD and develops predominantly as a result of episodic blood transfusions. This underscores the importance of the development and dissemination of evidence based guidelines, especially for episodic transfusions in SCD. A careful study of the extent and degree of organ damage associated with transfusional Fe overload in SCD and why less than half (46.2%) of patients are exposed to chelation therapy needs to be done. These studies should include liver iron concentration (LIC), cardiac iron and liver histology, when indicated, in parallel with serum hepcidin levels. The fact that the reduction in serum ferritin levels with deferasirox did not reach statistical significance in this cohort can be explained by the relatively small number of patients as well as by the short period (6 months) of exposure to chelation therapy. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Morbidity and mortality in chronically transfused subjects with thalassemia and sickle cell disease: A report from the multi-center study of iron overload

2007 ◽  
Vol 82 (4) ◽  
pp. 255-265 ◽  
Author(s):  
Ellen B. Fung ◽  
Paul Harmatz ◽  
Meredith Milet ◽  
Samir K. Ballas ◽  
Laura De Castro ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Iron Overload ◽  
Sickle Cell ◽  
Morbidity And Mortality ◽  
Cell Disease ◽  
Multi Center Study ◽  
Center Study

scholarly journals Average Monthly Cost of Iron Chelator Treatment in Adult Patients with Sickle Cell Disease

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 4858-4858
Author(s):  
Samip Master ◽  
Richard Preston Mansour
Keyword(s):  
Sickle Cell Disease ◽  
Iron Overload ◽  
Retrospective Analysis ◽  
Sickle Cell ◽  
Conflicts Of Interest ◽  
Private Insurance ◽  
Iron Chelator ◽  
Adult Patients ◽  
Cell Disease ◽  
Monthly Cost

Introduction: Iron overload in adult patients with sickle cell disease (SCD) can lead to variety of complications like liver dysfunction/cirrhosis, cardiac enlargement, diabetes mellitus, hypogonadism and arthropathy. These complication can be prevented by iron chelation therapy .We did retrospective analysis to find incidence of iron over load in this population and also did a survey to find the insurance status in this population. Methods: We take care of approximately 300 adult patients with SCD at out hematology clinic. We did retrospective analysis to investigate the prevalence of iron overload in this population. We also did survey on 100 adult patients with SCD to find out about the insurance converge for them. Web search was done to find out the average monthly cost of iron chelators. Results: On retrospective analysis of 458 adult patients with SCD, we found that 117/458(25.58%) had iron over load. Majority of them, 93/117 were SS type of SCD. Results of survey done on 100 adult patients with SCD showed that 61 had Medicaid, 2 were free care, 25 had Medicare and 12 had private insurance. The average monthly cost of Deferiprone is $ 18762, while that of Deferasirox is $ 13,082. Conclusions: Iron over load is a common complication affecting a quarter of the adult patients with SCD. The treatment of iron overload is expensive, as just the iron-chelator therapy costs approximately 160 to 220 K per year. In an attempt to minimize additional iron accumulation in our chronically transfused patient population we encourage the schedule of exchange of 1 unit phlebotomy and 1 unit of red cell infusion every two weeks. Disclosures No relevant conflicts of interest to declare.

scholarly journals Sickle Cell Disease and COVID-19 Infection: A Single-Center Experience

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 985-985
Author(s):  
Sindhu Devarashetty ◽  
Kimberly Le Blanc ◽  
Udhayvir Singh Grewal ◽  
Jacqueline Walton ◽  
Tabitha Jones ◽  
...  
Keyword(s):  
Pulmonary Embolism ◽  
Sickle Cell Disease ◽  
Mortality Rate ◽  
Sickle Cell ◽  
Adult Patients ◽  
Living Alone ◽  
Morbidity And Mortality ◽  
Acute Chest Syndrome ◽  
Single Center ◽  
Cell Disease

Abstract Background and Objectives: The COVID-19 (CO19) pandemic caused by SARS-CoV-2 remains a significant issue for global health, economics, and society. Several reports have shown that African Americans (AA) have been disproportionately affected by the CO19 pandemic. Limited data have suggested that sickle cell disease (SCD) could be one of the several reasons for higher morbidity and mortality related to CO19 among AA. Recent reports have suggested higher-than-average morbidity and mortality related to CO19 among patients with SCD. We conducted a retrospective, single-institution study in adult patients with SCD who were diagnosed with CO19 infection and their outcomes. Methods: After IRB approval, we conducted a chart review of adult patients (greater than 18 years) with SCD who were diagnosed with CO19 infection between March 1st, 2020, and March 31st, 2021. We recorded demographic data including age, gender, social factors (the type of insurance, availability of primary care provider (PCP), living alone/not), clinical parameters (type of SCD, co-morbidities), outpatient management of SCD, and how CO19 infection was managed like inpatient admission and complications. In patients who were admitted or seen in the emergency department (ED), we collected additional data including vitals, labs, the severity of illness, complications, length of stay, and outcomes. Computations were performed using statistical software SAS 9.4 for Windows. Results: We found a total of 51 patients with SCD diagnosed with CO19 infection in the above period. The median age of patients was 30 years. 61% were females and 39 % were males. All of them were AA. 11.76% were living alone, 49.02% were living with family, 1.96% (1 patient) was institutionalized, and the living situation was unknown in 37.25%. Most of the patients had Medicaid Insurance (52.94%), Medicare in 33.3%, private insurance in 13.73 % and 2% were uninsured. Only 64.71% of patients had a PCP. 60% had HbSS disease, 32% had HbSC disease, 4% had HbS-beta thalassemia, one patient each had HbSS with hereditary persistence of HbF and HbS/HbD. Comorbidities and previous history included acute chest syndrome in 65.96%, avascular necrosis in 36.96%, leg ulcers in 8.7%, hypertension in 8.7%, sickle cell retinopathy in 14.57%, cerebrovascular disease in 26.19%, chronic kidney disease in 7.69%, venous thromboembolism (VTE) in 20.41%, 10.41% were on anticoagulation, history of HIV and hepatitis C infection in 6.38%. 28.21% of patients were maintained on partial exchange transfusions as an outpatient for various indications. 72.73% were on hydroxyurea, 7.5% were on crizanlizumab, 5.26% were on voxelotor and 26.83% were on iron chelation. Vitals and pertinent lab values on initial assessment were recorded and many patients had missing data. On presentation, 25.53% were febrile, 29.17% of patients were tachycardic, 31.25% were hypoxic (SpO2 < 95%), 38.46% were tachypneic, 59.18% had a body mass index (BMI) of > 24.9. Median hemoglobin and hematocrit were 8.9/27.4 g/dL. The median white blood cell count was 9490/uL and platelets were 315,000/uL. Median ferritin was 1573 ug/L. Median bilirubin and creatinine were 2.05 mg/dL and 0.86 mg/dL. The patients were further stratified based on the clinical location where CO19 infection was managed (Table 1). 39.3% were diagnosed in the outpatient setting/ED and 60.3% in the inpatient setting. Among 51 patients, 5.71% (n=2) required ICU admission and was mechanically ventilated. 17.5% received dexamethasone, 7.69% received remdesivir, 2.76% received convalescent plasma, 17.07% had infections and 47% received antibiotics. Only one patient received an exchange transfusion during admission. One patient developed a new VTE after CO19 infection. On statistical analysis, the only factor which impacted the clinical location of management was tachycardia (P=0.007). Of the 51 patients, only 3.9% (2 patients) died of complications of CO19 infection, one with hypoxic respiratory failure, disseminated intravascular coagulation, shock, and the other one with pulmonary embolism. 13% were readmitted within a month, one of them was admitted with a new pulmonary embolism and the others were admitted for acute painful episodes. Conclusion: We found a mortality rate of 3.9% in our single-center study of patients with SCD and CO19 infection. This mortality rate is lower than other published experiences in patients with SCD and CO19 infection. Figure 1 Figure 1. Disclosures Master: Blue Bird Bio: Current holder of individual stocks in a privately-held company.

Hyperferritinemia and Hemophagocytic Lymphohistiocytosis. Single Institution Experience in Adult and Pediatric Patients.

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 2135-2135 ◽  
Author(s):  
Alice D. Ma ◽  
Yuri D. Fedoriw ◽  
Philip Roehrs
Keyword(s):  
Sickle Cell Disease ◽  
Iron Overload ◽  
Liver Failure ◽  
Sickle Cell ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Pediatric Patients ◽  
Adult Patients ◽  
Cell Disease ◽  
Clinical Criteria ◽  
Shock Liver

Abstract Abstract 2135 Hemophagocytic lymphohistiocytosis (HLH) is a multisystem disorder characterized by immune dysregulation and hypercytokinemia. Diagnostic criteria include a genetic mutation consistent with familial HLH or the presence of 5 of 8 defined clinical criteria (fever, splenomegaly, bicytopenia, hypertriglyceridemia and/or hypofibrinogenemia, hemophagocytosis, low/absent NK cell function, hyperferritinemia, and elevated soluble CD25). In pediatrics, a ferritin value of >10,000 mχγ/L has been reported to have 90% sensitivity and 96% specificity in defining the presence of HLH (Allen, C. E., Yu, X., Kozinetz, C. A. and McClain, K. L. (2008). Pediatr. Blood Cancer). We examined if hyperferritinemia (all patients with ferritin level >10,000 mχγ/L between 2007 and 2012) correlated with diagnosis of HLH in 94 patients (73 adult; 21 pediatric) at our institution. Chart reviews were performed to evaluate the presence or absence of HLH criteria, additional clinical features that may be indicative of HLH, and diagnosis. These data resulted in our classification of patients into four groups (Table 1): (1) “clinically defined HLH” when predetermined criteria were met; (2) “potential HLH” when clinical criteria was suggestive of HLH, but not all criteria were met; (3) “possible HLH” when rheumatologic syndromes, liver disease, or fever/DIC was present of unknown etiology; or (4) “Non-HLH” when the elevated ferritin was a result of a known etiology (Table 2). As expected, 18 (86 %) of pediatric patients with a ferritin > 10,000 mχγ/L had clinically defined or potential/possible HLH. Notably, 44 (60 %) of adult patients with a ferritin > 10,000 mg/L had clinically defined or potential/possible HLH. Such an incidence of HLH in the adult population with elevated ferritin raises caution for appropriate diagnosis of this population and clearly warrants further study. If patients with sickle cell disease, GVH, or known causes for liver failure are excluded, then HLH should be suspected in 83% of adult patients with ferritins >10,000 mcg/L. Table 1: HLH classifications of 94 patients with ferritin > 10,000 mχγ/L Adult, n = 73 n (%) Pediatric, n = 21 n (%) Clinically defined HLH 18 (25) 12 (57) Potential HLH 9 (12) 5 (24) Possible HLH 17 (23) 1 (5) Non-HLH 29 (40) 3 (14) Table 2: Diagnoses of non-HLH patients with ferritin > 10,000 mcg/L (some diagnoses overlap) Liver failure of clear etiology (10) APAP toxicity (4) Shock liver (4), EtOH, Other Sickle cell disease (9) 7 adult, 2 peds, all with probable iron overload Other tumors (9) CMML+VT+shock liver, prostate ca with bone mets, CMML to AML, MDS/MPD with infection, AML, T lymphoblastic lymphoma with cholestasis, allo txp for AML with iron overload, ALL+ abd wall hematoma Iron overload (11) 9 sickle cell, 1 Castlemans, 1 allo txp for AML Other Pancreatitis, GVH (3) Table 3: Clinical suspicion for HLH Potential HLH (%) Possible HLH (%) Total Adult 9 17 Peds 5 1 HLH suspected? Adult 1 (11) 2 (12) Peds 4 (80) 1 (100) All criteria sent? Adult 1 (11) 1 (6) Peds 2 (40) 1 (100) Hematology involved? Adult 6 (67) 11 (64) Peds 5 (100) 1 (100) Did hematologist note ferritin? Adult 1 (17) 3 (27) Peds 5 (100) 0 Disclosures: Ma: Novo Nordisk Inc.: Consultancy, Speakers Bureau.

scholarly journals Prevalence of Disease Related Complications from in Adult Patients with Sickle Cell Disease

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 4862-4862
Author(s):  
Samip Master ◽  
Shajadi Patan ◽  
Shashank Cingam ◽  
Richard Preston Mansour
Keyword(s):  
Sickle Cell Disease ◽  
Iron Overload ◽  
Renal Dysfunction ◽  
Retrospective Analysis ◽  
Sickle Cell ◽  
Health Science ◽  
Adult Patients ◽  
Acute Chest Syndrome ◽  
Leg Ulcers ◽  
Cell Disease

Abstract Introduction: Sickle cell disease can lead to variety of complications like strokes, acute chest syndrome (ACS), sickle cell hepatopathy, priapism, renal dysfunction, avascular necrosis (AVN) ofjoints , pulmonary hypertension and leg ulcers. Average life expectancy of patient with SCD has improved with better treatments available. Most of literature on SCD in form pediatric literature and data on adults is considerably lacking. We did a retrospective analysis to investigate the prevalence of complications in adult patients with SCD. Methods: We take care of around 300 adult active patients with SCD at our hematology clinic. We did a retrospective analysis from 458 adult patients seen at our clinic between 2001 and 2016. We collected data on gender, race, type of SCD, complications like stroke, acute chest syndrome, iron overload, leg ulcers, AVN, hepatopathy and renal dysfunction. Results: A total of 458 adult patients with SCD were analyzed. 231 were female and 227 were males. The total number of patients with SC disease was 114, SS was 263, sickle beta thal was 75 and 4 had SS with hereditary persistence of Hemoglobin F (SS HPF). The number patient with complication has been listed in table below. Conclusion: Adult Centers for the treatment of Sickle Cell Disease need to optimize care directed at these high frequency, life changing and life shortening complications. Multidisciplinary care teams have to focus on these chronic complications that include transfusion program, iron overload treatment and prevention, orthopedic care and Hepatitis C and B directed carealong with acute complication care. This retrospective study serves as the basis for our adult Sickle Cell Disease program at Louisiana State University Health Science Center in Shreveport, Louisiana. Table Table. Disclosures No relevant conflicts of interest to declare.

scholarly journals Poor Access to Care Is a Risk Factor for Iron Overload in Adults with Sickle Cell Disease in Southern California

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 3527-3527
Author(s):  
Susan Claster ◽  
Michael Roland ◽  
Jeri Tucker ◽  
Marlene Espinoza ◽  
Ellen Rothman ◽  
...  
Keyword(s):  
Risk Factor ◽  
Los Angeles ◽  
Sickle Cell Disease ◽  
Iron Overload ◽  
Access To Care ◽  
Sickle Cell ◽  
Southern California ◽  
Morbidity And Mortality ◽  
Beta Thalassemia ◽  
Cell Disease

Abstract The lack of access to quality comprehensive care is a major problem for adults with sickle cell disease (SCD) and contributes to increased morbidity and mortality. This problem is especially acute in Los Angeles where the average lifespan is 10 years shorter than the national average. Without access to disease modifying therapies, patients utilize Emergency Department services and are repeatedly hospitalized. During inpatient stays many may be inappropriately transfused. The result can be severe iron overload, a significant cause of morbidity and mortality. Inattention to iron chelation in patients who are not cared for by knowledgeable SCD providers perpetuates this issue. In 2016 we cared for a new patient at our clinic in South Los Angeles who previously had a liver transplant due iron overload. This patient received multiple transfusions during her lifetime and was never seen in a comprehensive SCD clinic. This experience prompted us to evaluate our population of clinic patients to assess how lack of access to care might be a risk factor for iron overload. Methods: We performed a chart review of all patients with SCD who presented to either of two adult sickle cell clinics in Southern California: MLK Jr. Outpatient Center in Los Angeles and Center for Inherited Blood Disorders (CIBD) in Orange. We examined records from 2013-2018 from CIBD and from 2016-2018 from MLK. Patients were considered evaluable if they had a least one visit to clinic with labs that included iron studies and /or MRI. Iron saturations greater than 50% and /or a ferritin greater than 1000 was deemed indicative of iron overload when combined with a history of prior transfusions. Lack of access was defined as the absence of appropriate care for SCD for at least 12 months prior to the initial visit in our clinics. Patients were deemed in good SCD care if they had been transitioned from a pediatric SCD program or another adult SCD program where they received team based comprehensive services. Results: 74 patients were able to be evaluated. (Table 1). 53 patients were Hgb SS. Of those, 27 (50.9%) were not receiving appropriate SCD care for the prior 12 months when they first presented, 20 (74.1%) of those patients were iron overloaded. There were 9 patients with Hgb S/beta thalassemia 0 or +. Five (55.6%) of those patients were not receiving appropriate SCD care for the prior 12 months; one (35.6%) was iron overloaded. 12 patients had Hgb SC. Although 5 (41.7%) were not receiving appropriate SCD care at the time of presentation, none had evidence of iron overload. Using these data, the odds ratio of having iron overload if a patient was out of appropriate SCD care for the 12 months prior to evaluation was 6.8 (95% confidence interval 2,23.13), indicating that being out of good care is a risk factor for iron overload. Discussion: Although guidelines exist to prevent unnecessary transfusions in adult patients with SCD; lack of access to knowledgeable providers both in the inpatient and outpatient setting places them at a nearly 7 times higher risk for iron overload, a serious and preventable comorbidity. Our study indicates that many adults with SCD are not appropriately monitored or treated for transfusion related iron overload using guideline-based care, placing avoidable burdens on individual health and adding to healthcare costs. Further education of providers to address this issue is warranted. Disclosures Baker: Genentech: Research Funding.

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