A pilot MRI study of organ specific hemosiderosis and functional correlation in Chinese patients with myelodysplasia and aplastic anemia with raised ferritin levels

Au, WY; Lam, Wwm; Chu, Winnie C.W.; Tam, Sidney; Wong, Wai Lap; Chan, Hoi Ming Herman; Law, Man Fai; Liu, Hsy; Liang, Raymond

doi:10.1002/hon.862

Cited by 11 publications

(8 citation statements)

References 21 publications

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“…Those who become transfusion dependent can develop substantial morbidity and significantly reduced overall and leukaemia-free survival (6)(7)(8)(9).…”

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confidence: 99%

Deferasirox treatment of iron‐overloaded chelation‐naïve and prechelated patients with myelodysplastic syndromes in medical practice: results from the observational studies eXtend and eXjange

Gattermann

Jarisch

Schlag

et al. 2011

European J of Haematology

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EXtend and eXjange were prospective, 1-yr, non-interventional, observational, multicentre studies that investigated deferasirox, a once-daily oral iron chelator, in iron-overloaded chelation-naïve and prechelated patients with myelodysplastic syndromes (MDS), respectively, treated in the daily-routine setting of office-based physicians. No inclusion or exclusion criteria or additional monitoring procedures were applied. Deferasirox was administered as recommended in the European Summary of Product Characteristics. Haematological parameters and adverse events (AEs) were collected at two-monthly intervals. Data from 123 chelation-naïve patients with MDS (mean age 70.4 yrs) with median baseline serum ferritin level of 2679 (range 184–16 500) ng/mL, and 44 prechelated patients with MDS (mean age 69.6 yrs) with median baseline serum ferritin level of 2442 (range 521–8565) ng/mL, were assessed. The mean prescribed daily dose of deferasirox at the first visit was 15.7 and 18.7 mg/kg/d, respectively. Treatment with deferasirox produced a significant reduction in median serum ferritin levels in chelation-naïve patients with MDS from 2679 to 2000 ng/mL (P = 0.0002) and a pronounced decrease in prechelated patients with MDS from 2442 to 2077 ng/mL (P = 0.06). The most common drug-related AEs were gastrointestinal, increased serum creatinine levels and rash. These studies demonstrate that deferasirox used in physicians’ medical practices is effective in managing iron burden in transfusion-dependent patients with MDS.

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“…Those who become transfusion dependent can develop substantial morbidity and significantly reduced overall and leukaemia-free survival (6)(7)(8)(9).…”

mentioning

confidence: 99%

Deferasirox treatment of iron‐overloaded chelation‐naïve and prechelated patients with myelodysplastic syndromes in medical practice: results from the observational studies eXtend and eXjange

Gattermann

Jarisch

Schlag

et al. 2011

European J of Haematology

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“…Increased iron absorption and subsequent increase liver iron has been described in 85% of patients with Hb H disease [16]. Despite high hepatic pancreatic and pituitary iron observed by MRI, cardiac hemosiderosis or overt endocrine dysfunction was uncommon [17]. A greater risk for iron-induced organ damage can be expected in Hb H-CS.…”

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confidence: 99%

Hemoglobin H‐constant spring in North America: An alpha thalassemia with frequent complications

Singer¹,

Kim

Olivieri

et al. 2009

American J Hematol

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Hemoglobin H-constant spring (Hb H-CS), the most common nondeletional alpha thalassemia in Asia is increasingly recognized in North America due to shifts in immigration patterns. In California, alpha (α)-thalassemia syndromes are the second most frequent finding among newborns screened for hemoglobinopathies with a two-fold increase compared to a decade earlier [1,2]. Though known to have a more severe anemia than Hb H disease, the other clinical findings of Hb H-CS are not well described. Moreover, beneficial therapies that have become available in the last decade are often not applied to their care. This analysis of 46 patients enrolled in the Thalassemia Clinical Research Network (TCRN) age 13+/− 10 years old, with Hb H-CS revealed moderate anemia (mean 8.7 ± 1.5 g/dl), regular transfusion therapy in 24% of patients, and splenomegaly or prior splenectomy in one-third of them. Serum transferin receptor (sTfr), was elevated; (44.4 ± 18 mcg/ml normal range 2.9–8.3 mcg/ml), reflecting ineffective erythropoiesis, which in turn leads to high iron absorption and increased ferritin levels in younger (median = 187 ng/ml) and older (median = 465 ng/ml) nontransfused patients. These findings along with moderate growth delay and low bone mass were more prevalent in Hb H-CS patients compared to deletional Hb H disease. Our results highlight the required monitoring of the extent of anemia, growth, splenomegaly, iron overload, gallstones, bone density and assessment of need for transfusions and specific treatments for disease complications.

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“…The clinical importance of iron overload in MDS and the risk‐benefit balance of iron chelation therapy are incompletely understood and controversial topics at present [29, 30]. There have been at least three prior publications assessing cardiac iron overload measured noninvasively using newer T2*/R2* MRI techniques in heavily transfused MDS populations, and these three studies all suggested that cardiac iron overload is infrequent and is less common than hepatic iron overload in heavily transfused patients with MDS [31–33]. The new GFM cardiac MRI study reported at ASH 2010 included a cohort of patients with a high median number of red cell units transfused (68 U), but so did the three previous studies: a median of 63 U in the 11‐patient United Kingdom study by Chacko et al, 90 U in the 10‐patient Israeli study by Konen et al, and 36 U in the 11‐MDS‐patient Chinese study by Au et al [31–33] It is notable that serum ferritin did not predict cardiac iron overload in any of these studies, which emphasizes the poor test characteristics of serum ferritin measurement.…”

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confidence: 99%

ASH 2010 meeting report—Top 10 clinically‐oriented abstracts in myelodysplastic syndromes (MDS)

Steensma

2011

American J Hematol

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The December 2010 American Society of Hematology (ASH) annual meeting in Orlando, Florida included more than 150 abstracts in the myelodysplastic syndromes (MDS) category. Thirty-six MDS-focused abstracts were presented in 6 oral sessions, and 120 additional abstracts were presented in 3 poster sessions. Although the 2010 ASH annual meeting included relatively little new MDS clinical trial data, with only one large randomized trial presented (the Intergroup E1905 study), it was an exciting conference from the standpoint of molecular discoveries in myeloid neoplasms, as various investigative groups presented results offering novel insights into MDS pathobiology, generated via high-throughput genomic sequencing and other innovative laboratory techniques. Here I summarize and discuss 10 MDS-related abstracts of special interest, which contain information that informs clinical practice. All 10 of these abstracts are published in full in the November 19, 2010 issue of Blood (volume 116, issue 21) and are identified here by their abstract number and title. Diagnosis and Prognosis No. 1Discrepancy in diagnosis of myelodysplastic syndrome (MDS) between referral and tertiary care centers: experience at MD Anderson cancer center (MDACC) (Abstract # 1870). Abstract summaryThe MD Anderson cancer center (MDACC) leukemia group reviewed data from 915 patients who presented to their institution in Houston, Texas between 2005 and 2009 carrying an outside diagnosis of myelodysplastic syndrome (MDS). Among these 915 patients, there was discordance in diagnosis between the referring practice and the referral center in 150 patients (16%). Sixty of the 150 patients had been diagnosed prior to MDACC referral with forms of MDS without excess marrow blasts [i.e., refractory anemia (RA), refractory anemia with ring sideroblasts (RARS), and refractory cytopenias with multilineage dysplasia (RCMD)]; 46 of these 60 were reassessed at MDACC as having refractory anemia with excess blasts (RAEB), and 6 were rediagnosed as RAEB in transformation (RAEB-t; these patients would be classified as having acute myeloid leukemia (AML) by World Health Organization (WHO) diagnostic criteria [1]). Fifteen patients diagnosed with RAEB on the outside were assessed as having lower-risk MDS at MDACC (i.e., with less than 5% blasts), while 40 patients diagnosed with RAEB on the outside were rediagnosed with RAEB-t. A handful of other patients labeled as MDS underwent diagnostic revision to one of the myeloproliferative neoplasms (MPN), MDS/MPN overlap syndromes, AML, or a benign disorder or indeterminate condition. Causes of diagnostic discrepancy included inadequate initial diagnostic material, differences in sample preparation and staining, over-reliance on flow cytometric assessment of blast proportion, and interpretive differences. While the changes in diagnosis did not have a significant global impact in terms of median survival or clinical outcome, new diagnoses did alter treatment recommendations, clinical trial eligibility, and prognosis for individual...

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A pilot MRI study of organ specific hemosiderosis and functional correlation in Chinese patients with myelodysplasia and aplastic anemia with raised ferritin levels

Cited by 11 publications

References 21 publications

Deferasirox treatment of iron‐overloaded chelation‐naïve and prechelated patients with myelodysplastic syndromes in medical practice: results from the observational studies eXtend and eXjange

Deferasirox treatment of iron‐overloaded chelation‐naïve and prechelated patients with myelodysplastic syndromes in medical practice: results from the observational studies eXtend and eXjange

Hemoglobin H‐constant spring in North America: An alpha thalassemia with frequent complications

ASH 2010 meeting report—Top 10 clinically‐oriented abstracts in myelodysplastic syndromes (MDS)

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