The myelodysplastic syndromes (MDS) are characterized by ineffective hematopoiesis associated with multilineage cytopenias leading to serious morbidity or mortality, and the additional risk of leukemic transformation. The management of patients with MDS can be very complex and varies according to both the clinical manifestations in individual patients as well as the presence of complicating medical conditions. However, therapeutic dilemmas still exist for MDS due to the multifactorial pathogenetic features of the disease, its heterogeneous stages, and the elderly patient population. For these reasons, proper guidelines for management are necessary. This review describes the proper diagnosis for MDS, decision-making approaches for optimal therapeutic options that are based on a consideration of patient clinical factors and risk-based prognostic categories, and the use of recently available biospecific drugs such as hypomethylating agents that are potentially capable of abrogating the abnormalities associated with MDS. Proper indications and methods for transplantation, response criteria, management for iron overload for highly transfused patients and specific considerations for MDS in childhood are also described. All of these topics were discussed at the third symposium of AML/MDS working party on 3 March, 2007.
Aged ; Diagnosis ; Hematopoiesis ; Humans ; Iron Overload ; Mortality ; Myelodysplastic Syndromes* ; Transplantation

OBJECTIVECompare the characteristics of magnetic resonance imaging（MRI）liver T2*, cardiac T2* and serum ferritin on the assessment of iron overload.

METHODSA total of sixty-nine patients from November 2011 to June 2014 were enrolled in this study. Their cardiac and liver iron concentration levels were measured through MRI examination, with other clinical data were collected to perform statistical analysis.

RESULTSThe correlation between liver T2* and adjusted serum ferritin（ASF） was statistically significant（P=0.003）. However, no significant correlation was found between cardiac T2* and liver T2*, ASF, respectively. According to the statistical analysis of the 69 cases, it is found that the number of iron overload cases diagnosed by liver T2* was 62 and 20 cases were severe iron overload （32.26%）; the number of iron overload cases diagnosed by ASF was 47 and 14 cases were severe iron overload（29.79%）, while the number of iron overload cases diagnosed by cardiac T2* was only 25 and no severe iron overload cases.

CONCLUSIONSince SF was affected by other factors, it cannot reflect the level of iron overload in human body objectively. Now, liver T2* has become the gold standard for assessment of iron overload because of its good reliability and repeatability. However, cardiac T2* cannot correctly be used as assessment for iron overload, and it is only a method of evaluating the level of cardiac iron deposition.

Ferritins ; blood ; Hematologic Diseases ; diagnosis ; Humans ; Iron Overload ; diagnosis ; Liver ; Magnetic Resonance Imaging ; Myocardium ; Reproducibility of Results

Humans ; Iron Overload ; diagnosis ; etiology ; therapy ; Myelodysplastic Syndromes ; diagnosis ; metabolism ; therapy

Magnetic induction method aims at the noninvasive detection of liver iron overload by measuring the hepatic magnetic susceptibility. To solve the difficulty that eddy current effects interfere with the measurement of magnetic susceptibility, we proposed an improved coil system based on the static field magnetization principle in this study. We used a direct current excitation to eliminate the eddy current effect, and a rotary receiver coil to get the induced voltage. The magnetic field for a cylindrical object due to the magnetization effect was calculated and the relative change of maximum induced voltage was derived. The correlation between magnetic susceptibility of object and maximum magnetic flux, maximum induced voltage and relative change of maximum induced voltage of the receiver coil were obtained by simulation experiments, and the results were compared with those of the theory calculation. The contrast shows that the simulation results fit the theory results well, which proves our method can eliminate the eddy current effect effectively.
Computer Simulation ; Humans ; Image Processing, Computer-Assisted ; Iron Overload ; diagnosis ; Liver ; physiopathology ; Magnetic Fields ; Magnetics ; instrumentation

BACKGROUND: In chronic renal failure (CRF) patients, iron deficiency is a common problem and a primary cause of resistance to recombinant human erythropoietin (rHuEPO) therapy. Serum ferritin and transferrin saturation (TS) are most commonly used parameters of iron status in CRF patients but may be influenced by the presence of inflammation and malnutrition. Recently soluble transfer-rin receptor (sTfR) has been advocated as a useful parameter of iron deficiency. We evaluated sTfR as an iron deficient marker in CRF patients. METHODS: Included in this study were 73 CRF patients, 30 uncomplicated iron deficiency anemia (IDA) patients, and 55 normal control. Serum sTfR, serum ferritin, TS, and complete blood count were measured. The CRF patients were classified as absolute iron deficient, functional iron deficient, non-iron deficient, and iron overload groups according to National Kidney Foundation Kidney Disease and Dialysis Outcome Quality Initiative (NKF-K/DOQI) guideline. RESULTS: The sTfR concentrations were significantly higher in uncomplicated IDA patients (3.9 +/-1.5 mg/L) and significantly lower in CRF patients (1.1 +/-0.4 mg/L) than in normal controls (1.4 +/-0.4mg/L). In uncomplicated IDA patients, sTfR was inversely correlated with MCV, MCH, and MCHC. In CRF patients, sTfR had a weak inverse correlation with TS and MCHC, but not significantly different between the four groups. The sTfR was not significantly different between the CRF patients with the normal CRP and those with an increased CRP. CONCLUSIONS: The sTfR is useful for diagnosis of uncomplicated IDA, but not for the detection of iron deficiency in CRF patients. Further studies are needed for the evaluation of sTfR as an erythro-poietic marker with rHuEPO therapy.
Anemia, Iron-Deficiency ; Blood Cell Count ; Diagnosis ; Dialysis ; Erythropoietin ; Ferritins* ; Humans ; Inflammation ; Iron ; Iron Overload ; Kidney ; Kidney Diseases ; Kidney Failure, Chronic* ; Malnutrition ; Receptors, Transferrin* ; Transferrin

BACKGROUND: Iron overload is a risk factor affecting all patients with thalassemia intermedia (TI). We aimed to determine whether there is a relationship of serum ferritin (SF) and alanine aminotransferase (ALT) with liver iron concentration (LIC) determined by R2 magnetic resonance imaging (R2-MRI), to estimate the most relevant degree of iron overload and best time to chelate in patients with TI. METHODS: In this cross-sectional study, 119 patients with TI (mean age years) were randomly selected and compared with 120 patients who had a diagnosis of thalassemia major (TM). Correlations of LIC, as determined by R2-MRI, with SF and ALT levels, were assessed in all participants. A P-value < 0.05 was considered statistically significant. RESULTS: SF and LIC levels were lower in patients with TI than in those with TM; only ferritin values were significant. We found a statistically significant relationship between SF and LIC, with cut-off estimates of SF in patients with TI who had splenectomy and those who entered puberty spontaneously (916 and 940 ng/mL, respectively) with LIC >5 mg Fe/g dry weight (P < 0.0001). A significant relationship was also found for patients with TI who had elevated ALT level (63.5 U/L), of 3.15 times the upper normal laboratory limit, using a cut-off for LIC ≥5 mg Fe/g dry weight. CONCLUSION: We determined the cut-off values for ALT and SF indicating the best time to start iron chelation therapy in patients with TI, and found significant correlations among iron overload, SF, and ALT.
Adolescent ; Alanine Transaminase ; beta-Thalassemia* ; Chelation Therapy ; Cross-Sectional Studies ; Diagnosis ; Ferritins* ; Humans ; Iron Overload ; Iron* ; Liver* ; Magnetic Resonance Imaging ; Puberty ; Risk Factors ; Splenectomy ; Thalassemia*

PURPOSE: We investigated the epidemiological and clinical characteristics of diabetes mellitus developed in aplastic anemia patients who have had many blood transfusions. METHODS: We retrospectively reviewed medical records of 170 patients with aplastic anemia who were diagnosed before 15 years of age in Department of Pediatrics, The Catholic University of Korea from 1987 to 2001. We obtained their medical history, family history of diabetes mellitus, clinical onset of diabetes mellitus and the successive history, and coexistence of other disorders. RESULTS: Diabetes mellitus was diagnosed in 8 of 107 patients (7.5%) with severe aplastic anemia in childhood. The mean age of diagnosis of diabetes mellitus was 18.5+/-5.2 years, and the mean duration from the start of blood transfusion to the diagnosis of diabetes mellitus was 7.7+/-2.9 years. Duration of multiple blood transfusions was a major risk factor for the development of diabetes mellitus in severe aplastic anemia patients. There was a wide range of symptoms at clinical onset of diabetes mellitus from asymptomatic hyperglycemia to diabetic ketoacidosis. Incidence of other complications, such as hepatic impairment (88%) and cardiac dysfunction (75%), was high in patients who developed diabetes mellitus. CONCLUSION: Severe aplastic anemia patients treated with prolonged, multiple transfusions have a significant risk of developing insulin-deficient diabetes mellitus. These patients are at risk for other complications, such as hepatic, cardiac, or thyroid disorders. Early prevention of iron overload and screening for transfusion-related complications are very important in these patients.
Anemia, Aplastic* ; Blood Transfusion ; Diabetes Mellitus* ; Diabetic Ketoacidosis ; Diagnosis ; Humans ; Hyperglycemia ; Incidence* ; Iron Overload ; Korea ; Mass Screening ; Medical Records ; Pediatrics ; Retrospective Studies ; Risk Factors ; Thyroid Gland

OBJECTIVETo assess the value of magnetic resonance imaging T2* tests in the detection of myocardial and liver iron overload in patients with β-thalassemia major (β-TM).

METHODSFrom 2010 to 2011, 28 β-TM patients over 10 years old under blood transfusion therapy and chelation care with serum ferritin (SF)>1000 µg/L underwent myocardial and liver MRI T2* tests on a voluntary basis. The results were analyzed in relation with age, SF, and left ventricular ejection fraction (LVEF).

RESULTSFourteen out of the 28 cases (50%) were found to have myocardial iron overload, including 7 severe cases, 2 moderate cases, and 5 mild cases. All the 28 cases had liver iron overload, including 2 mild cases, 7 moderate cases, and 19 severe cases. Two out of the 28 cases had lowered LVEF (7.14%), and one of them had severe myocardial iron overload. There was a negative correlation between myocardial MRI T2* and SF (r=-0.479, P=0.01). Myocardial MRI T2* was positively correlated with liver MRI T2* (r=0.378, P=0.047). Age was not significantly correlated with SF, LVEF, or liver MRI T2*.

CONCLUSIONMagnetic resonance imaging (T2*) detection is an effective and non-invasive means for detecting myocardial and liver iron overload in patients with β-thalassemia major receiving blood transfusion. T2* combined with SF is the main diagnostic indicator to assess iron overload in the vital organs.

Adolescent ; Adult ; Child ; Female ; Ferritins ; blood ; Humans ; Iron ; metabolism ; Iron Overload ; diagnosis ; metabolism ; pathology ; Liver ; metabolism ; Magnetic Resonance Imaging ; Male ; Myocardium ; metabolism ; Young Adult ; beta-Thalassemia ; diagnosis ; metabolism ; pathology

OBJECTIVE: To evaluate the diagnostic performance of magnetic resonance elastography (MRE) for staging hepatic fibrosis in patients with chronic hepatitis B virus (HBV) infection. MATERIALS AND METHODS: Patients with chronic HBV infection who were suspected of having focal or diffuse liver diseases (n = 195) and living donor candidates (n = 166) underwent MRE as part of the routine liver MRI examination. We measured liver stiffness (LS) values on quantitative shear stiffness maps. The technical success rate of MRE was then determined. Liver cell necroinflammatory activity and fibrosis were assessed using histopathologic examinations as the reference. Areas under the receiver operating characteristic curve (Az) were calculated in order to predict the liver fibrosis stage. RESULTS: The technical success rate of MRE was 92.5% (334/361). The causes of technical failure were poor wave propagation (n = 12), severe respiratory motion (n = 3), or the presence of iron deposits in the liver (n = 12). The mean LS values, as measured by MRE, increased significantly along with an increase in the fibrosis stage (r = 0.901, p < 0.001); however, the mean LS values did not increase significantly along with the degree of necroinflammatory activity. The cutoff values of LS for > or = F1, > or = F2, > or = F3, and F4 were 2.45 kPa, 2.69 kPa, 3.0 kPa, and 3.94 kPa, respectively, and with Az values of 0.987-0.988. CONCLUSION: MRE has a high technical success rate and excellent diagnostic accuracy for staging hepatic fibrosis in patients with chronic HBV infection.
Adolescent ; Adult ; Aged ; *Elasticity Imaging Techniques ; Female ; Hepatitis B, Chronic/complications/*diagnosis ; Humans ; Iron Overload/diagnosis ; Liver Cirrhosis/*diagnosis/pathology ; Living Donors ; Male ; Middle Aged ; Movement ; ROC Curve ; Respiration ; Young Adult