scholarly journals Red Blood Cell Transfusion Independence Following the Initiation of Iron Chelation Therapy in Myelodysplastic Syndrome

2010 ◽  
Vol 2010 ◽  
pp. 1-5 ◽  
Author(s):  
Maha A. Badawi ◽  
Linda M. Vickars ◽  
Jocelyn M. Chase ◽  
Heather A. Leitch
Keyword(s):  
Time Course ◽  
Chelation Therapy ◽  
Transfusion Requirement ◽  
Iron Chelation ◽  
Hemoglobin Level ◽  
Red Blood Cell Transfusion ◽  
Iron Chelation Therapy ◽  
Transfusion Independence ◽  
Transfusion Requirements ◽  
Rbc Transfusion

Iron chelation therapy is often used to treat iron overload in patients requiring transfusion of red blood cells (RBC). A 76-year-old man with MDS type refractory cytopenia with multilineage dysplasia, intermediate-1 IPSS risk, was referred when he became transfusion dependent. He declined infusional chelation but subsequently accepted oral therapy. Following the initiation of chelation, RBC transfusion requirement ceased and he remained transfusion independent over 40 months later. Over the same time course, ferritin levels decreased but did not normalize. There have been eighteen other MDS patients reported showing improvement in hemoglobin level with iron chelation; nine became transfusion independent, nine had decreased transfusion requirements, and some showed improved trilineage myelopoiesis. The clinical features of these patients are summarized and possible mechanisms for such an effect of iron chelation on cytopenias are discussed.

scholarly journals Red Blood Cell Transfusion Independence Following the Initiation of Iron Chelation Therapy in Myelodysplastic Syndrome.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 4844-4844
Author(s):  
Maha A Badawi ◽  
Linda M Vickars ◽  
Jocelyn M Chase ◽  
Heather A Leitch
Keyword(s):  
Platelet Count ◽  
Myelodysplastic Syndrome ◽  
Research Funding ◽  
Chelation Therapy ◽  
Ferritin Level ◽  
Iron Chelation ◽  
Iron Chelation Therapy ◽  
Transfusion Independence ◽  
Transfusion Requirements ◽  
Rbc Transfusion

Abstract Abstract 4844 Background Iron chelation therapy (ICT) is often used to treat iron overload (IOL) in patients (pts) requiring transfusion of red blood cells (RBC) for chronic anemia. In myelodysplastic syndrome (MDS), guidelines recommend consideration of ICT in pts with lower risk International Prognostic Scoring System (IPSS) and IOL as defined by a ferritin level >1000 ug/l; IOL related organ dysfunction; or receipt of ≥20 RBC units. During treatment of a pt with MDS and IOL with ICT, RBC transfusion requirement (TR) ceased. Here we report his course and review reported cases of RBC transfusion independence (TI) or decreased RBC TR in MDS pts receiving ICT. Methods The pt chart was reviewed and reported cases identified by PubMed search using the terms ‘MDS’ and ‘iron chelation’. The clinical characteristics and course of published cases were summarized. Case A 76 year (y) old man was referred in May 2004 for management of MDS diagnosed in 1997, when the white blood cell (WBC) count was 2.4 ×109/l; neutrophils, 0.7 ×109/l; hemoglobin (Hb), 133 g/l; platelets, 108 ×109/l. Bone marrow aspiration and biopsy showed refractory anemia (RA), karyotype analysis 46,X,-Y,+8, and the IPSS score was intermediate-1. The erythropoitin (epo) level was 148.3 mIU/ml and the stem cell assay showed no epo-independent colony growth. In 2004 the Hb dropped to 60 g/l prompting the initiation of RBC transfusion support. He required 3 RBC units every 4 weeks to maintain a Hb >90 g/l and complained of fatigue and functional limitation. Creatinine, bilirubin, TSH, reticulocyte count, B12 and folate levels were all normal. The ferritin level in 2004 was 1293 ug/l and 2197 ug/l in 2006. He declined ICT with deferoxamine (DFO) but in 2006 accepted deferasirox (DFX). He required several dose interruptions and adjustments for renal insufficiency; the current dose is 5mg/kg/d with a normal creatinine. Two months (mo) after starting ICT, the Hb increased spontaneously to 109 g/l and he has not required RBC transfusion since. The mean Hb since starting ICT was 122 g/l and the ferritin decreased to 1082 ug/l in 2009. The most recent neutrophil count was 3.5 ×109/l, platelets consistently clump and the MCV is unchanged at 120 fl. He reports excellent energy and an improved quality of life, and has remained clinically well and RBC transfusion independent to the present, 36 mo from the initiation of ICT. Literature review There are 18 published cases of MDS showing improvement in Hb with ICT; 9 became RBC transfusion independent. Characteristics of the 10 TI pts were: median age at MDS diagnosis 58 (range 18-74) y; male, n=5. MDS subtype: RA, n=5; RARS, n=2, RCMD, n=1; RAEB, n=2. IPSS (reported in 8): low, n=1; int-1, n=5; int-1 or 2, n=1; high, n=1. ICT was: DFO, n=7; DFX, n=3. Median time to RBC TI was 17.5 (1-24) mo and TI duration 13 (3-28) mo to date. Of pts who had decreased RBC transfusion requirements with ICT but did not achieve transfusion independence: median age (reported in 3) was 67 (45-78) y; gender (reported in 3) female, n=3; MDS subtype: RA, n=8; RAEB-t, n=1; IPSS: int-1, n=3; ICT: DFO, n=8; DFX, n=1. Median time to decreased TR was 14.4 (3-24) mo; median duration of decreased TR (reported in 3) 9 (6-32) mo; initial TR 50.9 (19.7-447) g Hb/mo; median decrease in TR 12.7 (0.1-88) g Hb/mo. In one report of 6 pts, 2 with pancytopenia showed improvement with ICT in WBC from 1.4 to 1.9 ×109/l (p<0.0001) and neutrophils from 0.51 to 0.94 ×109/l (p<0.001). The platelet count increased from 16.6 to 22.5 ×109/l (p<0.001) and 14.6 to 29.6 ×109/l (p<0.00001) within 3 mo and the MCV decreased significantly in 5 by a mean of 5.1 (2.1-11.7) fl, normalizing in 2. In a second report, neutrophils increased in 8 of 9 pts; in 4 the initial neutrophil count was <1 ×109/l, and platelet counts increased in 7 of 11 pts, in 4 the initial platelet count was <20 ×109/l. Conclusions In summary, our pt is the 19th patient with MDS reported to date in whom improved Hb followed the initiation of ICT; 9 had a decrease in RBC transfusion requirements, and RBC transfusion independence occurred in 10. The remarkable course of these pts adds to evidence that ICT may be of clinical benefit for selected patients with MDS and IOL. Although the improvement in WBC and platelet counts with ICT in some pts implies a suppressive effect of IOL on hematopoiesis that may be abrogated by ICT, the mechanism by which the effects of ICT on transfusion requirements occur, and the frequency with which they occur, remains an area for future investigation. Disclosures Off Label Use: This presentation discusses the use of iron chelation therapy deferoxamine and deferasirox in patients with myelodysplastic syndrome.. Vickars:Novartis Canada: Honoraria, Research Funding. Leitch:Novartis Canada: Honoraria, Research Funding, Speakers Bureau.

scholarly journals Durable Red Blood Cell Transfusion Independence in a Patient with an MDS/MPN Overlap Syndrome Following Discontinuation of Iron Chelation Therapy

2015 ◽  
Vol 2015 ◽  
pp. 1-7 ◽  
Author(s):  
Harpreet Kochhar ◽  
Chantal S. Leger ◽  
Heather A. Leitch
Keyword(s):  
Blood Cell ◽  
Iron Overload ◽  
Red Blood Cell ◽  
Chelation Therapy ◽  
Iron Chelation ◽  
Red Blood Cell Transfusion ◽  
Favorable Effect ◽  
Iron Chelation Therapy ◽  
Transfusion Independence ◽  
Rbc Transfusion

Background. Hematologic improvement (HI) occurs in some patients with acquired anemias and transfusional iron overload receiving iron chelation therapy (ICT) but there is little information on transfusion status after stopping chelation.Case Report. A patient with low IPSS risk RARS-T evolved to myelofibrosis developed a regular red blood cell (RBC) transfusion requirement. There was no response to a six-month course of study medication or to erythropoietin for three months. At 27 months of transfusion dependence, she started deferasirox and within 6 weeks became RBC transfusion independent, with the hemoglobin normalizing by 10 weeks of chelation. After 12 months of chelation, deferasirox was stopped; she remains RBC transfusion independent with a normal hemoglobin 17 months later. We report the patient’s course in detail and review the literature on HI with chelation.Discussion. There are reports of transfusion independence with ICT, but that transfusion independence may be sustained long term after stopping chelation deserves emphasis. This observation suggests that reduction of iron overload may have a lasting favorable effect on bone marrow failure in at least some patients with acquired anemias.

Clinical Management of Myelodysplastic Syndromes With Interstitial Deletion of Chromosome 5q

2006 ◽  
Vol 24 (16) ◽  
pp. 2576-2582 ◽  
Author(s):  
Stephen D. Nimer
Keyword(s):  
Myelodysplastic Syndromes ◽  
Chelation Therapy ◽  
Iron Chelation ◽  
Normal Karyotype ◽  
Transfusion Independence ◽  
Chromosome 5Q ◽  
Transfusion Requirements ◽  
Definition Of ◽  
Classification And Treatment ◽  
Rbc Transfusion

Deletions of the long (q) arm of chromosome 5 [del(5q)]occur in patients with myelodysplastic syndromes (MDS) including, but not limited to, those who meet the WHO definition of the 5q− syndrome. Del(5q) MDS patients frequently have symptomatic anemia, and its treatment has traditionally consisted of RBC transfusions and, for some, iron chelation therapy. Erythropoietin, darbepoetin, hypomethylating agents, and lenalidomide can enhance erythropoiesis in MDS patients with anemia, increasing hemoglobin levels and abrogating RBC transfusion requirements. Lenalidomide is particularly active in treating the anemia of del(5q) MDS, which is especially relevant given the low response rate to erythropoietin in this group of patients. In a recent study of 43 MDS patients, 10 of 12 patients (83%) with del(5q) MDS achieved sustained RBC transfusion independence (or a > 2 g/dL increase in hemoglobin), compared with 57% of those with a normal karyotype and 12% of those with other karyotypic abnormalities. Complete cytogenetic remissions were achieved in 75% (nine of 12) of the del(5q) MDS patients, suggesting that lenalidomide targets a fundamental pathogenetic feature of MDS that is more pronounced in the presence of chromosomal 5q deletions. This review highlights some issues about the classification and treatment of del(5q) MDS.

RBC Transfusions and Iron Chelation Therapy in Clinical Practice in MDS: A One Month Survey by the GFM.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 2661-2661
Author(s):  
Christian Rose ◽  
A. Stamatoulas ◽  
D. Vassilief ◽  
S. Brechignac ◽  
O. Beyne-Rauzy ◽  
...  
Keyword(s):  
Chelation Therapy ◽  
Transfusion Requirement ◽  
Iron Chelation ◽  
Skin Lesions ◽  
Median Number ◽  
Iron Chelation Therapy ◽  
Duration Of Treatment ◽  
Positive Effects ◽  
Median Serum ◽  
Rbc Transfusion

Abstract Background: RBC transfusions remain a mainstay in the treatment in MDS, but their characteristics in a large MDS population have not been reported in detail. Indications of iron chelation therapy (CT) in those patients, are based on consensus (Gattermann Hematology/Oncology Clinics 2005;19:supp1 ) but have not been reported in a large MDS cohort. In addition, a positive effect of desferioxamine (DFO) therapy on hematopoiesis has been reported in a small group of MDS (Jensen BJH, 1996;94:288). Methods: We analyzed the hematological characteristics, RBC transfusion requirement and iron CT in MDS patients referred for RBC transfusion in 18 French centers between May 15 and June 15, 2005. Results: 170 pts were included in the survey, median age 75 (14–95); M/F ratio 1.37; median time from diagnosis was 43 months (1 month to 17.5 years); WHO: pure RA 8.8%, pure RARS 18%, RCMD 3.5%, RCMD-RS 3%, RAEB I 16.5%, RAEB II 5.3%, 5q- syndrome 7%, CMML 4.7%, unclassified12.6%. Karyotype: fav (12%), int (37%), unfav ( 23%), failure or not done ( 28%). IPSS: low 39%, Int1 45%, Int2 13.5%, high 2.5%, unavailable 28%. Median serum ferritin (SF) level at diagnosis was 562ng/ml (9–2500). Median number of RBC units transfused since diagnosis was 49, mean 80 (2–610) and did not differ statistically according to WHO or IPSS. 81% of patients had received > 21 RBC units. Median number of RBC units transfused at initiation of CT was 21 (2–151). 68/170 pts ( 40 %) had received CT during the disease course including: DFO continuous s/c (8h) (40mg/kg/d, 3 to 5d/ week) n = 37, DFO s/c bolus (2 to 8g/week) n = 14, DFO IV (50 to 100mg/kg/d once after each RBC transfusion) n=7, deferiprone alone (30 to 75 mg/kg/d) n=4. Deferiprone + DFO s/c n=6. Median SF level was 1492ng/ml at the start of iron CT (436–6572). 50/68 pts were still on iron CT at the time of the survey after 2 to 114 months. Median SF level at the time of survey was 1828ng/ml (not statistically different from pre CT level). No positive effects were observed on hematopoiesis even in pts chelated for prolonged periods. 18/68 patients stopped CT (all were on DFO therapy) due to over efficacy n= 1, local complications n = 7, patient decision n=10 ). Median duration of treatment was 9 months (1–78) and median level of SF 3227ng/ml at the time of survey in those 18pts. For the 102/170 pts who did not receive CT, main reasons given by physicians were: older age 33% (median age in this group was indeed 82); cumbersome treatment 12%; high IPSS 8% (all cases had indeed IPSS>=1.5); low transfusion requirement 8% (median=11 RBC units transfused in this group); other reasons 5% (refusal, skin lesions), no reason given 34%. By comparison to non chelated patients, chelated patients had significantly higher number of RBC units transfused (mean 115 vs 55) (p<0.001), lower age (p= 0.002) but no difference in WHO or IPSS. Conclusions: In transfused MDS pts, rarely analyzed, the number of RBC units previously transfused was not influenced by WHO or IPSS. This survey confirms that chelated patients are younger and more transfused. Despite heterogeneity in iron CT, efficacy based on the SF level seems to be good. No effect of iron CT on hematopoiesis was observed.

Positive Impact of Iron Chelation Therapy (CT) on Survival in Regularly Transfused MDS Patients. A Prospective Analysis by the GFM.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 249-249 ◽  
Author(s):  
Christian Rose ◽  
Sabine Brechignac ◽  
Dominique Vassilief ◽  
Odile Beyne-Rauzy ◽  
A. Stamatoullas ◽  
...  
Keyword(s):  
Chelation Therapy ◽  
Transfusion Requirement ◽  
Positive Impact ◽  
Iron Chelation ◽  
Prospective Analysis ◽  
Iron Chelation Therapy ◽  
Kaplan Meier ◽  
Survival Difference ◽  
Impact On Survival ◽  
Rbc Transfusion

Abstract Background: RBC transfusions are required in most MDS, leading to iron overload which probably contributes to shortened survival (Malcovati, JCO, 2005). Consensus for indications of iron chelation therapy (CT) in MDS patients are recent (Gattermann Hemato/Oncol Clin2005; 19:supp1). A positive impact on survival of CT, clearly demonstrated in thalassemia, has not yet been prospectively reported in large MDS cohorts. Methods: We performed in 2005 a survey on hematological data, RBC transfusion requirement and CT in 170 MDS referred for RBC transfusion during a month period (May 15–June 15, 2005) to 18 French GFM centers (Rose, ASH 2006, abstr:2661). Survival of this prospective cohort was reanalyzed at the reference date of May 15, 2007. Results: 5 pts were lost to follow up. Median age of the remaining 165 pts was 77 (range 14–95).M/F 1.4. WHO: 13 pure RA (10%), 30 pure RARS (23%), 6 RCMD (5%),5 RCMD-RS (4%), 28 RAEB I (21%), 10 RAEB II (8%),11 5q- Sd (7%), 8 CMML (6%); 21 MDS unclass (16%). Karyotype: fav (12%), int (36%), unfav (22%), failure or ND (30%) IPSS: low 27%, Int1 32%, Int2 10%, high 2%, unavailable 29%. 76 pts (46%) received CT for at least 6 months, including 65 before May 2005, and 11 since May 2005. Median interval from diagnosis to onset of CT was 30 months (range 0–192). CT included: DFO continuous s/c (8h) (40mg/kg/d, 3 – 5d/w) n= 41, deferiprone alone (30 to 75 mg/kg/d) n= 5, Deferiprone + DFO s/c n= 5, deferasirox (20 to 30 mg/kg/d) n= 6 (Defined as “standard” chelation) and DFO s/c bolus (2 to 3g/week) n = 12 or DFO IV (50 to 100mg/kg/d once after each RBC transfusion) n= 7 (defined as “low” chelation). Median duration of CT was 35 months (6–138+). Median serum ferritin (SF) level was 569ng/ml (range 9–2500), 1436ng/ml (range 436–6572) and 1498ng/ml (range 272–7502) at diagnosis, onset of CT and last evaluation, respectively (resp). By comparison to non chelated pts, chelated pts had significantly higher number of RBC units transfused (mean 104 vs 57) (p&lt;0.001), lower age (mean 70 vs 76) (p= 0.006) no difference in WHO classification (p=0.274) but differences in IPSS: IPSS 0; 0–1,&gt;1 in 27%, 53%, 20% of non chelated versus 49%,36%,15% of chelated pts with available IPSS, resp (p=0.044). Median overall survival from diagnosis (OS, Kaplan-Meier analysis) was 115 and 51 months in chelated and non chelated pts,resp (p&lt; 0.0001). After adjustment on other prognostic parameters (sex,age, IPSS, transfusion requirement) the survival difference remained significant. In IPSS 0 pts, median OS was not reached in chelated pts and 69 months in non chelated pts(p=0.002).In IPSS 0–1 pts, median OS was 115 months in chelated pts and 50 months in non chelated pts (p=0.003). Longer interval from diagnosis to onset of CT did not influence OS negatively but median OS was 120 months with “standard” chelation versus 69 months with “low “chelation (p&lt;0.001). Conclusions: This prospective analysis strongly suggests that chelation therapy (CT) provides survival benefit in heavily transfused, mainly low and int 1, MDS. Confirmatory randomized trials, theoretically required,(CT vs no CT) may be difficult to perform in this situation.

Transfusion-Related Iron Overload: A Covert Risk Of Supportive Cancer Care

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 3656-3656
Author(s):  
Anushka Jaffer ◽  
Rebecca Barty ◽  
Erin Jamula ◽  
Grace Wang ◽  
Yang Liu ◽  
...  
Keyword(s):  
Iron Overload ◽  
Cancer Patients ◽  
Chelation Therapy ◽  
Iron Chelation ◽  
Iron Chelator ◽  
Adult Patients ◽  
Iron Chelation Therapy ◽  
Not Given ◽  
Screening Practices ◽  
Rbc Transfusion

Abstract Background Chronic transfusion support plays a key role in survival and quality of life for patients with hematological disorders. However, transfusion-related iron overload (TRIO) is a significant cause of morbidity and mortality in these patients.Adequate iron overload (IO) screening and use of iron chelators, if necessary, is now standard practice in chronically transfused individuals such as hemoglobinopathy patients. Screening practices for IO for patients receiving multiple red blood cell (RBC) transfusions for other reasons (e.g. cancer) are unknown. Objective This two part study aimed to detect pediatric (Jaffer et al., 2012) and adult populations at risk for TRIO and to evaluate and compare current screening practices. Methods Children (≤ 18 years) and adults (> 18 years) receiving at least 1 RBC transfusion from January 1, 2008 to December 31, 2011 at a tertiary care academic institution were identified using a transfusion registry database. Only those receiving chronic RBC transfusions were included in the study. Chronic transfusion was defined as ≥20 units of RBC or ≥ 20 RBC transfusions dosed at 15ml/kg within 12 consecutive months where transfusions were not given in the setting of an operating room, trauma or surgical procedures, not given 7 days prior/post-surgical procedures and not all given in one day. An adjudicator resolved study inclusion ambiguity. The analysis excluded hemoglobinopathy patients. Medical records were reviewed to collect patient demographics, diagnosis, and to evaluate IO screening practices and frequency of iron chelation therapy. Results A total of 343 patients met the eligibility criteria: 27 pediatric and 316 adult patients, with mean ages of 8.1 years (SD 5.7) and 62 years (SD 12.6), respectively. Table 1 summarizes demographics, number of transfusions, and IO screening and results. Ferritin levels were checked for 12 (44%) pediatric and 227 (72%) adult patients: 2 (17%) pediatric and 30 (13%) adult patients had values<500 μg indicating no further TRIO screening was required. In the pediatric population, 81% had a cancer diagnosis, and just under a third were tested for ferritin, whereas 64% of the adults had cancer, with nearly two-thirds tested for ferritin. A statistically significant difference was observed in the percentage of pediatric and adult cancer patients screened for IO. Of those cancer patients screened, ferritin level > 500 occurred in 71% of pediatric and 85% of adult patients, with an iron chelator reported in 1 adult. Total RBC transfusions ranged from 20 to 44 with a median of 26.5 for pediatrics and 20 to 176 with a median of 31 for adults. Conclusion Despite high rates of RBC transfusion, screening for TRIO was inconsistent. Although information regarding reasons for not screening for TRIO or not treating with chelation therapy was not collected, the possibilities include a lack of awareness of the risk of TRIO and lack of access to ferriscan and/or to oral iron chelator in Canada for conditions other than hemoglobinopathy and a select subset of MDS cases. Considering TRIO presents an additional, yet unidentified, co-morbidity of cancer therapy and that the therapy (e.g. anthracyclines) may potentiate the end organ effect of TRIO, it is vital to develop strategies to evaluate cancer patients at risk for TRIO and ensure they have access to appropriate iron chelation therapy. Research is needed to explore the comorbidities associated with failure to treat TRIO and to identify barriers to treatment so cancer patients can receive optimal care. Disclosures: Leber: Novartis Canada: Membership on an entity’s Board of Directors or advisory committees, Speakers Bureau. Heddle:Canadian Blood Services and Health Canada: Research Funding.

scholarly journals A case of transfusion independence in a patient with myelodysplastic syndrome using deferasirox, sustained for two years after stopping therapy

2015 ◽  
Vol 22 (2) ◽  
pp. 128 ◽  
Author(s):  
D. Sanford ◽  
C.C. Hsia
Keyword(s):  
Myelodysplastic Syndrome ◽  
Iron Overload ◽  
Chelation Therapy ◽  
Iron Chelation ◽  
Iron Chelator ◽  
Red Blood Cell Transfusion ◽  
Transfusion Independence ◽  
Clinical Complications ◽  
Increased Risk ◽  
History Of

Patients with myelodysplastic syndrome (mds) experience clinical complications related to progressive marrow failure and have an increased risk of developing acute myeloid leukemia. Frequent red blood cell transfusion can lead to clinical iron overload and is associated with decreased survival in mds patients. Iron chelation therapy reduces markers of iron overload and prevents end-organ damage.Here, we present the case of a patient with lowrisk mds with transfusional iron overload. He was treated for 2 years with an oral iron chelator, deferasirox, and after 12 months of treatment, he experienced a hemoglobin increase of more than 50 g/L, becoming transfusion-independent. He has remained transfusion-independent, with a normal hemoglobin level, for more than 2 years since stopping chelation therapy. Hematologic and erythroid responses have previously been reported in mds patients treated with iron chelation. The durability of our patient’sresponse suggests that iron chelation might alter the natural history of mds in some patients.

Are Children with Cancer Receiving Myelosuppressive Therapy At Risk for Transfusion-Related Iron Overload?

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 1180-1180
Author(s):  
Anushka Jaffer ◽  
Rebecca Barty ◽  
Erin Jamula ◽  
Grace Wang ◽  
Yang Liu ◽  
...  
Keyword(s):  
At Risk ◽  
Iron Overload ◽  
Chelation Therapy ◽  
Ferritin Level ◽  
Iron Chelation ◽  
Iron Chelation Therapy ◽  
Children With Cancer ◽  
Screening Practices ◽  
The Impact ◽  
Rbc Transfusion

Abstract Abstract 1180 Background Transfusion-related iron overload (TRIO) is associated with significant morbidity and mortality. Adequate screening for iron overload (IO) and the use of iron chelators, if needed, should be employed for chronically transfused individuals. However, with the exception of patients with hemoglobinopathies, screening for iron overload is not a consistent part of routine care for patients receiving multiple red cell transfusions, and is not identified as a treatable problem. Objective This study aimed to identify the population at risk for TRIO and to evaluate current screening practices. Methods All children (≤ 18 years) receiving at least one red blood cell (RBC) transfusion between January 1, 2008 and December 31, 2011 at our institution were identified using the TRUST (Transfusion Registry for Utilization, Surveillance and Tracking) database. Only patients receiving chronic RBC transfusion were included in this study, which was defined as receiving ≥20 units of RBC or ≥ 20 RBC transfusions dosed at 15ml/kg within 12 consecutive months where the transfusions were not administered in the setting of an operating room, trauma or surgical procedure(s), not administered 7 days prior/post-surgical procedures and not all administered within one day. Adjudication by a second reviewer resolved any ambiguity regarding study inclusion. Medical records of eligible patients were reviewed to collect patient demographics, underlying diagnosis and reason for transfusions, and to evaluate IO screening practices (e.g. ferritin level, testing for systemic IO (e.g. FerriScan) if persistently high ferritin) and frequency of iron chelation therapy. Results A total of 35 patients fulfilled the eligibility criteria, with a mean age of 8.82 years (SD 5.36). Table 1 summarizes the demographics of the population, the transfusion requirements, how often the patient subgroups were screened and the screening results. In summary, 20 patients had ferritin levels checked, where 2 (AML and hepatoblastoma) patients had values under 500 μg and no screening was required. Of the remaining 18, 10 patients were diagnosed with a hemoglobinopathy (8) and congenital anemia (2) requiring chronic transfusions and underwent regular screening for iron overload and received iron chelation therapy. The remaining 8 patients had ferritin level >500 μg but no IO screening ordered. Of these 8 patients the majority were diagnosed with a cancer (leukemia, solid tumours) (5), acquired aplastic anemia (2), and hemophagocytic syndrome (1). The total number of transfusions for these 8 patients ranged from 20 to 52 with a median of 25 transfusions. Conclusion The majority (63%) of chronically transfused patients in this cohort had underlying cancer requiring aggressive chemotherapy. Only 32% of these patients had ferritin level tested and none were evaluated for systemic IO. TRIO may represent an additional, as yet unidentified, co-morbidity of cancer therapy. Therapies such as anthracycline or radiation may potentiate the end organ effect of TRIO at levels lower than that observed in patients with a hemoglobinopathy. Hence, it is important to develop strategies to evaluate children with cancer at risk for IO and to study the impact of transfusional iron accumulation on end organ function. Disclosures: No relevant conflicts of interest to declare.

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