Magnetic Resonance Imaging (MRI) Detection of Iron Overload In Patients with Myelodysplastic Syndrome (MDS),

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 3827-3827
Author(s):  
Fabio P S Santos ◽  
Claudia Bley ◽  
Ricardo Helman ◽  
Guilherme Fleury Perini ◽  
Leandro de Padua Silva ◽  
...  

Abstract Abstract 3827 Introduction: Transfusion dependent anemia and iron overload (IO) are associated with reduced survival in MDS. Serum ferritin is the most common method of assessing body iron content, but is also an inflammatory marker, and may not correlate with IO in specific organs. T2* magnetic resonance imaging (MRI) is a non-invasive method for detecting IO. The prevalence of IO in patients with MDS, as detected by T2* MRI, is currently unknown. Purpose: We designed a single center trial to evaluate the efficacy of T2* MRI in detection of IO in patients with MDS, determine the prevalence of iron overload in this disease and correlate MRI findings with iron indexes (ferritin, transferrin and labile plasma iron [LPI]). Material and methods: Patients with WHO-2008 defined MDS or chronic myelomonocytic leukemia (CMML), independent of transfusion requirements, were eligible. Patients receiving iron chelation therapy were excluded. Iron indexes were measured at the time of T2* MRI evaluation. Hepatic iron overload (HIO) was considered in patients with a hepatic iron concentration (HIC) ≥ 2 g/mg. Cardiac iron overload (CIO) was considered in patients with a T2* value < 20 milliseconds. Results: A total of 58 patients with MDS and two patients with CMML have been recruited. Three patients were not evaluated by MRI due to claustrophobia, so 57 patients remain for the analysis. Median age was 66 years (range 18–89). MDS subtypes by WHO included: refractory anemia (N=5), refractory anemia with ring sideroblasts (RARS; N=7), 5q- syndrome (N=4), refractory cytopenias with multilineage dysplasia (N=21), refractory anemia with excess blasts-I (N=7) and –II (N=7), unclassifiable MDS (N=3), CMML (N=2) and therapy-related MDS (N=1). Clinical features at time of MRI are presented in table 1. Median cardiac T2* value was 42 ms (range 19.7–70.1 ms), and only one patient had a T2* value indicative of CIO. Median HIC value was 3.9 g/mg (range 0.9–16 g/mg), and 68% of patients had HIO. Patients with HIO had higher ferritin levels (1182 ng/mL vs. 185 ng/mL, p<0.0001) and transferrin saturation (76% vs. 34%, p<0.0001), but no difference in LPI (0.13 vs. 0.12 mM, p=0.49). HIO was found in 76% of transfusion dependent patients, but in also 59% of patients without history of transfusions. Among patients with HIO but without a history of transfusions, 50% had RARS. Considering MRI as a gold standard for detecting HIO, a ferritin value ≥ 1.000 ng/ml had a positive predictive value (PPV) for HIO of 98% and a NPV of 48%; for transferrin saturation ≥ 50%, PPV was 91% and NPV was 55%. Conclusions: This is one of the largest studies evaluating IO in MDS by T2* MRI. The prevalence of iron overload in MDS is underestimated by using conventional iron indexes and T2* MRI can help in the early detection of IO. In some subtypes of MDS, such as RARS, mechanisms other than transfusion dependency lead to IO. Disclosures: Santos: Janssen-Cilag: Consultancy, Speakers Bureau; United Medical: Consultancy, Speakers Bureau; Novartis: Research Funding. Hamerschlak:Novartis: Research Funding.

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 3639-3639
Author(s):  
Alessia Pepe ◽  
Laura Pistoia ◽  
Nicola Romano ◽  
Giuseppe Colaci ◽  
Aurelio Maggio ◽  
...  

Abstract Background: Non-transfusion-dependent thalassemia (NTDT) is a term used to indicate patients who do not require lifelong regular transfusions for survival. Morbidity in NTDT patients is more common and serious than previously recognized. This study aimed to examine the association of age with the presence of iron overload assessed by Magnetic Resonance Imaging (MRI) and cardiovascular and endocrine complications in NTDT patients. Methods: We considered 170 patients with thalassemia intermedia never transfused o who received occasional transfusions consecutively enrolled in the Myocardial Iron Overload in Thalassemia (MIOT) project. Iron overload was quantified by the T2* Magnetic Resonance Imaging (MRI) technique. All complications were classified according to international guidelines. Results: Six groups of patients were identified: age<18 years (N=7), age 18-30 years (N=36), age 30-40 years (N=48), age 40-50 (N=45), age 50-60 years (N=26), and age≥60 years (N=8). The Table shows the comparison among the groups. The youngest patient showing hepatic iron (MRI liver iron concentration-LIC>3 mg/g dw) had 9 years of age and the frequency of hepatic iron did not significantly increase with age. Only one patient showed cardiac iron (global heart T2*<20 ms). Diabetes appeared only in patients with more than 50 years and showed a trend toward increasing with increasing age. Hypothyroidism and osteoporosis were not present in pediatric patients and were not associated to age. Hypogonadism was not present in patients with less than 30 years and its frequency was comparable among the age groups. No patient showed hypoparathyroidism. Only patients older than 30 years showed a cardiac complication (heart failure or arrhythmias), but the rate did not significantly increase with increasing age. Conclusions: Our data in NTDT are indicative of high rate of liver iron overload at early age and extremely rare cardiac iron overload. Endocrine or cardiac complications were not present in pediatric patients but in adult patients the frequency did not increase with advancing age. Table. Table. Disclosures Pepe: Chiesi Farmaceutici S.p.A., ApoPharma Inc., and Bayer: Other: No profit support.


Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 4960-4960
Author(s):  
Fabio PS Santos ◽  
Claudia Bley ◽  
Ricardo Helman ◽  
Guilherme Fleury Perini ◽  
Iracema Esteves ◽  
...  

Abstract Abstract 4960 Introduction: Transfusion dependent anemia and iron overload are associated with reduced survival in patients with MDS. Increased iron absorption at the gastrointestinal tract may also contribute to iron overload. Serum ferritin is the most common method of assessing body iron content, but it can be elevated in patients with inflammatory conditions, and may not correlate with iron overload in specific organs such as the heart. T2* MRI is a non-invasive method for detecting iron overload in patients with transfusion-dependent anemia, and its efficacy has been validated in patients with thalassemia major. There are few studies reporting on the efficacy of T2* MRI for detection of iron overload in patients with MDS. Objective: To evaluate the efficacy of T2* MRI in detection of iron overload in patients with MDS, the prevalence of iron overload in this disease and correlate MRI findings with iron indexes (ferritin, transferrin and non-transferrin bound iron [NTBI]). Methods: Patients with MDS or chronic myelomonocytic leukemia (CMML), independent of transfusion requirements, were recruited into a prospective, single center trial to assess the efficacy of T2* MRI for detection of iron overload in this scenario. Patients receiving iron chelation therapy were excluded. Iron indexes were measured at the time of T2* MRI evaluation. Hepatic iron overload was considered in patients with a hepatic iron concentration (HIC) ≥ 2 g/mg. Cardiac iron overload was considered in patients with a T2* value < 20 milliseconds. Mann-Whitney and Fischer exact tests were used to compare baseline continuous and categorical variables among patients with and without iron overload as assessed by HIC. Correlation between HIC and iron indexes was assessed with Spearman correlation. Results: A total of 37 patients with MDS and one patient with CMML were recruited. Three patients were not evaluated by MRI due to claustrophoby, so 35 patients remain for the analysis. Median age was 68 years (range 18–84). MDS subtypes by the WHO classification include refractory anemia (N=3), refractory anemia with ring sideroblasts (N=5), 5q- syndrome (N=3), refractory cytopenias with multilineage dysplasia (N=13), refractory anemia with excess blasts-I (N=6) and –II (N=3) and unclassifiable MDS (N=1). Information about transfusion requirement was available for 28 patients, and 14 (50%) were transfusion dependent. Twenty-two patients could be classified by the WHO Prognostic Score System (WPSS) and were categorized as very low-risk (N=6), low-risk (N=3), intermediate risk (N=6) and high risk (N=7). Median ferritin, transferrin saturation and NTBI values were 1079.6 ng/mL (range 21.8–12738 ng/mL), 63% (range 6–100%) and 0.34 microM (range 0–12.93 microM), respectively. Median cardiac T2* value was 45.3 ms (range 19.7–70.1 ms), and only one patient had a T2* value indicative of cardiac iron overload. Median HIC value was 3.31 g/mg (range 0.2–9.97 g/mg), and 66% of patients had hepatic iron overload. Patients with hepatic iron overload had higher ferritin levels (1181 ng/mL vs. 131 ng/mL, p=0.007) and transferrin saturation (64% vs. 39%, p=0.02), but no differences in NTBI (0.29 microM vs. 0.22 microM, p=0.42). Patients with elevated HIC had a higher prevalence of transfusion dependency but the difference was not significant (50% vs. 33%, p=0.67). Ferritin levels and transferrin saturation correlated with HIC (r = 0.552, p=0.001 [ferritin]; r = 0.609, p=0.001 [transferrin saturation]). Conclusion: T2* MRI can detect iron overload in patients with MDS. Iron overload in MDS cannot be solely explained by transfusion dependent anemia. The study is currently ongoing and updated results will be presented at the meeting. Disclosures: No relevant conflicts of interest to declare.


2019 ◽  
Vol 1 ◽  
pp. 2-6
Author(s):  
Asad Naqvi ◽  
Timothy Ariyanayagam ◽  
Mir Akber Ali ◽  
Akhila Rachakonda ◽  
Hema N. Choudur

Objective: The objective of this study was to outline a novel unique concept of secondary impingement of the muscles, myotendons, and tendons of the rotator cuff from hypertrophy as a result of strength training exercises. Methods: In this retrospective observational study, 58 patients were referred for an magnetic resonance imaging (MRI) by the orthopedic surgeon to the radiology department over a period of 1½ years. All patients gave a history of strength training exercises and presented with clinical features of rotator cuff impingement. Results: We identified features of hypertrophy of rotator cuff muscles, myotendons, and tendons in 12 of these 58 patients. This was the only abnormality on MRI. The hypertrophy of rotator cuff muscles and tendon bulk completely filling the subacromial space to the point of overfilling and resulting in secondary compressive features. Conclusion: Rotator cuff impingement is a common phenomenon that can occur with various inlet and outlet pathological conditions. However, rotator cuff impingement may also result from muscle and tendon hypertrophy from strength training regimens. Hypertrophy of the rotator cuff can result in overfilling of the subacromial space, leading to secondary impingement, which we have termed as “pseudo-impingement.”


Author(s):  
Hongzhang Zhu ◽  
Shi-Ting Feng ◽  
Xingqi Zhang ◽  
Zunfu Ke ◽  
Ruixi Zeng ◽  
...  

Background: Cutis Verticis Gyrata (CVG) is a rare skin disease caused by overgrowth of the scalp, presenting as cerebriform folds and wrinkles. CVG can be classified into two forms: primary (essential and non-essential) and secondary. The primary non-essential form is often associated with neurological and ophthalmological abnormalities, while the primary essential form occurs without associated comorbidities. Discussion: We report on a rare case of primary essential CVG with a 4-year history of normal-colored scalp skin mass in the parietal-occipital region without symptom in a 34-year-old male patient, retrospectively summarizing his pathological and Computer Tomography (CT) and magnetic resonance imaging (MRI) findings. The major clinical observations on the CT and MR sectional images include a thickened dermis and excessive growth of the scalp, forming the characteristic scalp folds. With the help of CT and MRI Three-dimensional (3D) reconstruction techniques, the characteristic skin changes could be displayed intuitively, providing more evidence for a diagnosis of CVG. At the 5-year followup, there were no obvious changes in the lesion. Conclusion: Based on our observations, we propose that not all patients with primary essential CVG need surgical intervention, and continuous clinical observation should be an appropriate therapy for those in stable condition.


1994 ◽  
Vol 67 (796) ◽  
pp. 339-341 ◽  
Author(s):  
S Bondestam ◽  
A Lamminen ◽  
V-J Anttila ◽  
T Ruutu ◽  
P Ruutu

Hematology ◽  
2017 ◽  
pp. 1-7 ◽  
Author(s):  
Pustika Amalia Wahidiyat ◽  
Felix Liauw ◽  
Damayanti Sekarsari ◽  
Siti Ayu Putriasih ◽  
Vasili Berdoukas ◽  
...  

2019 ◽  
Vol 18 (4) ◽  
pp. 255-258
Author(s):  
Naureen Abdul Khalid ◽  
◽  
Nainal Shah ◽  

Spinal epidural haematoma is a rare condition, which may be due to trauma, surgery, epidural catheterisation or disorders of coagulation. We report a case of 60 year old lady who was on warfarin for Atrial fibrillation (AF) presented with history of non-traumatic sudden onset pain in both legs and difficulty in walking. Magnetic resonance imaging (MRI) spine demonstrated epidural haematoma which was treated conservatively. Another dilemma was anticoagulation for AF. We examine the options to manage such case.


2014 ◽  
Vol 27 (02) ◽  
pp. 155-158 ◽  
Author(s):  
U. Geissbühler ◽  
P. Karli ◽  
F. Forterre ◽  
E. Linon

SummaryA two-year-old female Lucerne Hound was presented with a one-week history of signs of progressive neck pain, inappetence, apathy, and an elevated rectal temperature. Findings of magnetic resonance imaging (MRI) were consistent with a foreign body abscess in the epidural space at the level of the first and second cervical vertebrae. A leftsided dorso-lateral atlantoaxial approach was performed, revealing an epidural abscess containing a grass awn. The clinical signs resolved within three days of surgery and the dog made a full recovery. This case report shows that grass awns can migrate to the atlantoaxial region in dogs and MRI findings lead to a suspicion of caudo-cranial migration within the spinal canal.


2004 ◽  
Vol 118 (6) ◽  
pp. 450-452 ◽  
Author(s):  
Chul Ho Jang ◽  
Young Ho Kim

This paper reports an unusual case in which aseptic meningitis presented with sudden sensorineural hearing loss (SSNHL) associated with intralabyrinthine haemorrhage (ILH). A seven-year-old girl presented with sudden right-sided hearing loss with dizziness. She did not have a previous history of bleeding disorders. This child was assessed using audiograms and magnetic resonance imaging (MRI). The patient's hearing loss was irreversible. Steroid therapy was not effective. SSNHL associated with ILH can be one of the negative prognostic factors in children.


Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 1331-1331
Author(s):  
Victoria J Tindell ◽  
Victoria T Potter ◽  
Rachel Kesse-Adu ◽  
Laura Reiff-Zall ◽  
Aloysius Y Ho ◽  
...  

Abstract Abstract 1331 Several groups have identified iron overload, in terms of raised pre-transplant serum ferritin levels, as an independent adverse prognostic factor for patients undergoing myeloablative HSCT. While serum ferritin has been used as a common marker in clinical studies to evaluate the impact of iron overload following allogeneic transplantation, there are limitations to its use with it being an acute phase reactant, as well as its lack of specificity for predicting end-organ toxicities. Patients undergoing HSCT for bone marrow failure (BMF) syndromes usually have a significant red cell transfusion history, and although the majority of these patients receive non-myeloablative HSCT regimens, it is unclear as to the impact of iron overload in these patients on subsequent transplant outcomes. In order to address these questions, we performed a prospective study evaluating the pre-transplant serum ferritin together with concurrent T2* cardiac magnetic resonance imaging (MRI) and R2 liver MRI in 18 patients with BMF syndromes undergoing allogeneic HSCT. The diagnosis of the patients included MDS (RCMD/hypoplastic MDS) =10, acquired aplastic anaemia =7, fanconi anaemia =1. The median age of the patients at transplantation was 42 years, and all patients received a T-cell depleted non-myeloablative HSCT. All patients were transfusion dependent pre-HSCT, with a median number of red cell transfusions of 45 (range: 8–115). Pre-HSCT ferritin was performed within 2 weeks of HSCT, and the results were correlated with albumin and C-reactive protein to reduce the impact of ferritin as an acute phase reactant. T2* and R2 MRI were similarly performed within 2 weeks of HSCT. The median pre-HSCT ferritin was significantly raised at 2119 ug/l(range: 559–12235). In contrast, the T2* cardiac MRI was normal for all but one patient who had evidence of mild cardiac iron overload. All patients had a corresponding cardiac echocardiogram performed with an ejection fraction within normal limits. For the liver T2* MRI, 7 patients had evidence of none or mild hepatic iron overload, while 11 patients had moderate to severe iron overload. There was no correlation between pre-HSCT transfusion burden and serum ferritin levels. Furthermore, there was no correlation between either the transfusion burden or serum ferritin, and the T2*MRI readings. In terms of HSCT outcome, the median time to neutrophil engraftment was 14 days. 2 patients had primary graft failure and only 1 patient died within 100 days due to an intra-cerebral haemorrhage. No patients had any clinical features of hepatic veno-occlusive disease (VOD), and 5 patients had evidence of grade I-II acute grade versus-host-disease. Data were also collected on the incidence of bacterial, fungal and viral infections post-HSCT for the cohort. There was however no significant association between transfusion burden, serum ferritin or T2* imaging and any of the HSCT outcomes (engraftment/day 100 TRM, GvHD, VOD or infections). In the context of heavily transfused BMF patients receiving allogeneic HSCT, serum ferritin does not correlate with end-organ deposition of iron. Despite the high transfusion burden in our cohort of patients, cardiac deposition of iron appears minimal while hepatic iron deposition is significant in a large proportion of patients. Reassuringly, a raised iron overload by either of the above mentioned parameters had no effect on HSCT outcomes. Our findings highlight the limitations of using serum ferritin as a marker of iron overload pre-HSCT. The role of active pre-HSCT chelation of BMF patients receiving non-myeloablative HSCT regimens remains unclear, and further studies are warranted. Disclosures: No relevant conflicts of interest to declare.


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