Publications by authors named "Regine Grosse"

21 Publications

  • Page 1 of 1

Benefits of a Disease Management Program for Sickle Cell Disease in Germany 2011-2019: The Increased Use of Hydroxyurea Correlates with a Reduced Frequency of Acute Chest Syndrome.

J Clin Med 2021 Sep 30;10(19). Epub 2021 Sep 30.

Hopp-Children's Cancer Center (KiTZ), Department of Pediatric Oncology, Hematology and Immunology, Heidelberg, University of Heidelberg, Im Neuenheimer Feld 430, 69120 Heidelberg, Germany.

Sickle Cell Disease (SCD) is the most common monogenic disorder globally but qualifies as a rare disease in Germany. In 2012, the German Society for Paediatric Oncology and Haematology (GPOH) mandated a consortium of five university hospitals to develop a disease management program for patients with SCD. Besides other activities, this consortium issued treatment guidelines for SCD that strongly favour the use of hydroxyurea and propagated these guidelines in physician and patient education events. In order to quantify the effect of these recommendations, we made use of claims data that were collected by the research institute (WIdO) of the major German insurance company, the (AOK), and of publicly accessible data collected by the Federal Statistical Office (). While the number of patients with SCD in Germany increased from approximately 2200 in 2011 to approximately 3200 in 2019, important components of the recently issued treatment guidelines have been largely implemented. Specifically, the use of hydroxyurea has more than doubled, resulting in a proportion of approximately 44% of all patients with SCD being treated with hydroxyurea in 2019. In strong negative correlation with the use of hydroxyurea, the frequency of acute chest syndromes decreased. Similarly, the proportion of patients who required analgesics and hospitals admissions declined. In sum, these data demonstrate an association between the dissemination of treatment guidelines and changes in clinical practice. The close temporal relationship between the increased use of hydroxyurea and the reduction in the incidence of acute chest syndrome in a representative population-based analysis implies that these changes in clinical practice contributed to an improvement in key measures of disease activity.
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http://dx.doi.org/10.3390/jcm10194543DOI Listing
September 2021

Introduction of Universal Newborn Screening for Sickle Cell Disease in Germany-A Brief Narrative Review.

Int J Neonatal Screen 2021 Jan 28;7(1). Epub 2021 Jan 28.

Zentrum für Geburtshilfe, Kinder- und Jugendmedizin, Universitätsklinikum Eppendorf, Klinik und Poliklinik für Pädiatrische Hämatologie und Onkologie, Martinistr. 52, 20246 Hamburg, Germany.

Sickle cell disease (SCD) is a severe non-malignant disorder of hemoglobin and is inherited in an autosomal-recessive manner [...].
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http://dx.doi.org/10.3390/ijns7010007DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7931038PMC
January 2021

Investigating the suitability of high-resolution mass spectrometry for newborn screening: identification of hemoglobinopathies and β-thalassemias in dried blood spots.

Clin Chem Lab Med 2020 04;58(5):810-816

Newborn Screening and Metabolic Diagnostics Unit, Center of Diagnostics, University Medical Center Hamburg, Hamburg, Germany.

A fast and reliable method for the determination of hemoglobinopathies and thalassemias by high-resolution accurate mass spectrometry (HRAM/MS) is presented. The established method was verified in a prospective clinical study (HRAM/MS vs. high-pressure liquid chromatography [HPLC]) of 5335 de-identified newborn samples from the Hamburg area. The analytical method is based on a dual strategy using intact protein ratios for thalassemias and tryptic digest fragments for the diagnosis of hemoglobinopathies. Due to the minimal sample preparation and the use of flow injection, the assay can be considered as a high-throughput screening approach for newborn screening programs (2 min/sample). Using a simple dried blood spot (DBS) extraction (tryptic digest buffer), the following results were obtained: (1) a carrier incidence of 1:100 newborns (35 FAS, nine FAC, eight FAD and two FAE), and (2) no homozygous affected patient was detected. Using the HRAM/MS protocol, an unknown Hb mutation was identified and confirmed by genetic testing. In addition to greater specificity toward rare mutations and β-thalassemia, the low price/sample (1-2€) as well as an automated data processing represent the major benefits of the described HRAM/MS method.
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http://dx.doi.org/10.1515/cclm-2019-0832DOI Listing
April 2020

Sickle cell disease in Germany: Results from a national registry.

Pediatr Blood Cancer 2020 04 22;67(4):e28130. Epub 2019 Dec 22.

NCT Trial Center, National Center for Tumor Diseases (NCT) Heidelberg, German Cancer Research Center (dkfz), Heidelberg, Germany.

Background: Limited data on the prevalence and medical care of sickle cell disease (SCD) in Germany are available. Here, we make use of a patient registry to characterize the burden of disease and the treatment modalities for patients with SCD in Germany.

Procedure: A nationwide German registry for patients with SCD documents basic data on diagnosis and patient history retrospectively at the time of registration. A prospective annual documentation provides more details on complications and treatment of SCD. For the current analyses, data of 439 patients were available.

Results: Most patients had homozygous SCD (HbSS 75.1%, HbS/β-thalassemia 13.2%, and HbSC 11.3%). The median age at diagnosis was 1.9 years (interquartile range, 0.6-4.4 years), most patients were diagnosed when characteristic symptoms occurred. Sepsis and stroke had affected 3.2% and 4.2% of patients, respectively. During the first year of observation, 48.3% of patients were admitted to a hospital and 10.1% required intensive care. Prophylactic penicillin was prescribed to 95.6% of patients with homozygous SCD or HbS/β thalassemia below the age of six and hydroxycarbamide to 90.4% of patients above the age of two years. At least one annual transcranial Doppler ultrasound was documented for 74.8% of patients between 2 and 18 years.

Conclusion: With an estimated number of at least 2000, the prevalence of SCD in Germany remains low. Prospectively, we expect that the quality of care for children with SCD will be further improved by an earlier diagnosis after the anticipated introduction of a newborn screening program for SCD.
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http://dx.doi.org/10.1002/pbc.28130DOI Listing
April 2020

Serum ferritin is not a reliable predictor to determine iron overload in thalassemia major patients post-hematopoietic stem cell transplantation.

Eur J Haematol 2018 Dec 25;101(6):791-797. Epub 2018 Oct 25.

Department for Children and Adolescents, Division for Stem Cell Transplantation and Immunology, University Hospital Frankfurt, Frankfurt am Main, Germany.

Objective: Iron overload (IO) in transfusion-dependent anemia persists after hematopoietic stem cell transplantation (HSCT) and can cause long-term organ damage. In many studies, the diagnosis of IO before and after HSCT is based on serum ferritin (SF) levels rather than on assessment of liver iron concentration (LIC) by MRI or SQUID.

Method: In a retrospective multicenter study, we analyzed the concordance for indication of iron depletion therapy and correlation between LIC and SF of 36 thalassemia patients after HSCT. LIC was determined either by MRI-R2 (FerriScan®) or SQUID.

Results: The concordance between LIC and SF varies over time after transplant (P = 0.011). The correlation between SF and LIC was strong in the first year (Spearman's rho 0.75; P < 0.001). In agreement, the concordance between SF and LIC concerning indication for treatment was close to 1 with an overall error rate ca. of 10%. In particular in the first year after HSCT, SF underestimates the degree of iron overload. However, in the longitudinal analysis since the second year post-HSCT onward no association was found between LIC and SF (P = 0.217). Furthermore, in the second year after HSCT, the overall error rate was 35%, whereas in the 3rd, 4th, and >4th year, it was 58%, 60%, and 25%, respectively.

Conclusions: Our data suggest serum ferritin is not a reliable predictor to determine iron overload in thalassemia patients after HSCT.
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http://dx.doi.org/10.1111/ejh.13169DOI Listing
December 2018

The epidemiology of sickle cell disease in Germany following recent large-scale immigration.

Pediatr Blood Cancer 2017 Jul 6;64(7). Epub 2017 Apr 6.

Department of Pediatric Oncology, Hematology and Immunology, University of Heidelberg, Heidelberg, Germany.

Background: The epidemiology of sickle cell disease (SCD) in Germany is currently changing fundamentally with ongoing immigration. Here, we address the challenges resulting from the increased frequency, that is, the morbidity, and mortality of SCD in this population.

Procedure: The number of immigrants with SCD was estimated based on the data of the German central registry of migrants (2007-2015) and published epidemiologic data. Additional data analysis was based on nationwide aggregated data from the diagnosis-related groups' (DRG) statistics of the German Federal Statistical Office.

Results: The total number of patients with SCD among migrants was estimated at 2,016 in 2007 and 3,216 in 2015, thus showing a 60% increase, which was particularly remarkable during 2014 and 2015. The countries of origin included those of West sub-Saharan Africa, followed by Syria, and other countries of the Middle East. In parallel, the number of SCD inpatient treatments increased from 780 in 2002 to 1,340 in 2015. Between 2012 and 2014, 42 patients with SCD died in hospital, mostly at an age of less than 5 years (n = 7) or over 30 years (n = 29).

Conclusion: More than 3,000 patients with SCD are estimated to live among the immigrant population in Germany. In addition, the number of SCD patients of German nationality is not known. The increasing number of inpatient treatments and the death of young children from SCD indicate the need for a general newborn screening program and an increased awareness of this disease among medical practitioners in a country in which SCD used to be rare.
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http://dx.doi.org/10.1002/pbc.26550DOI Listing
July 2017

Building a National Framework for Adolescent and Young Adult Hematology and Oncology and Transition from Pediatric to Adult Care: Report of the Inaugural Meeting of the "AjET" Working Group of the German Society for Pediatric Oncology and Hematology.

J Adolesc Young Adult Oncol 2017 Jun 16;6(2):194-199. Epub 2016 Dec 16.

12 University Hospital Muenster, Westfalian Wilhelms University Muenster , Muenster, Germany .

Adolescents and young adults (AYAs) with hemato-oncological problems constitute a heterogenous group with characteristic particularities, specific needs, and age-related clinical and unique psychosocial features. Strong collaboration between pediatric and adult hemato-oncology settings is essential to address their needs appropriately. This is not only true for patients who first become ill during adolescence or young adulthood, but equally so for people who contract hemato-oncological diseases congenitally or as younger children and who are now becoming old enough to leave the pediatric setting and have to transit into "adult" medical care. Efforts to create environments that meet the specific needs of the AYA population affected by hemato-oncological diseases have been initiated in many countries. Due to international variations between societies in general and healthcare infrastructures in particular, the challenges posed to creating such environments vary considerably from country to country. Aiming at addressing these on a national basis for Germany, a dedicated Working Group on Adolescents, Young Adults, and Transition (Arbeitsgemeinschaft Adoleszenten, junge Erwachsene, Transition, AjET) was established. This meeting report depicts the content and discussions of the first interdisciplinary conference on treatment, transition, and long-term follow-up in AYAs with cancer or chronic/inborn hematological diseases. The AjET group of the German Society for Pediatric Oncology and Hematology (GPOH) intends to increase the national awareness for AYAs; strengthen the collaboration of pediatric and adult care givers; and initiate, promote, and coordinate collaborative activities in the fields of basic and translational research, clinical care, and long-term follow-up aimed at improving the current situation.
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http://dx.doi.org/10.1089/jayao.2016.0075DOI Listing
June 2017

Analysis of a cohort of 101 CDAII patients: description of 24 new molecular variants and genotype-phenotype correlations.

Br J Haematol 2016 Nov 29;175(4):696-704. Epub 2016 Jul 29.

Department Internal Medicine III, University Hospital Ulm, Ulm, Germany.

Congenital dyserythropoietic anaemia type II (CDAII) is a rare autosomal recessive disease characterized by ineffective erythropoiesis, haemolysis, erythroblast morphological abnormalities, hypoglycosylation of some red blood cell membrane proteins, particularly band 3, and mutations in the SEC23B gene. We report the analysis of 101 patients from 91 families with a median follow-up of 23 years (range 0-65); 68 patients are newly reported. Clinical and haematological parameters were separately analysed in early infancy and thereafter, when feasible. Molecular analysis of the SEC23B gene confirmed the high heterogeneity of the defect, leading to the identification of 54 different mutations, 24 of which are newly described. To evaluate the genotype-phenotype correlation, patients were grouped according to their genotype (two missense mutations vs. one missense/one drastic mutation) and assigned to two different severity gradings based on laboratory data and on therapeutic needs; by this approach only a weak genotype-phenotype correlation was observed in the analysed groups.
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http://dx.doi.org/10.1111/bjh.14271DOI Listing
November 2016

Left atrial active contractile function parameters assessed by cardiac MR are sensitive to myocardial iron.

J Magn Reson Imaging 2017 02 26;45(2):535-541. Epub 2016 Jul 26.

University Medical Center Hamburg-Eppendorf, Department of Diagnostic and Interventional Radiology, Hamburg, Germany.

Purpose: To determine the impact of myocardial iron overload on left atrial (LA) volume and function using MR in patients with systemic iron overload.

Materials And Methods: Thirty-eight patients with systemic iron overload disease and 10 controls underwent 1.5 Tesla MR performing steady state free precession short-axis cine-series of the LA. Three-dimensional-volumetry was assessed to calculate LA volumes and function. Parameters were indexed (i) to body surface area. The myocardial transverse relaxation rate R2* was determined in the ventricular septum using a multi-echo GRE sequence (breathhold; electrocardiography triggered; 12 echoes; echo time = 1.3-25.7 ms).

Results: Significantly decreased active atrial emptying fraction (AAEF) (23% [95%-range, 7-34] versus 36% [95%-range, 14-49], P = 0.009), active atrial emptying volume (AAEVi) (5.5 mL/m [95%-range, 2-11] versus 11.9 mL/m [95%-range, 3-23], P = 0.008), and active peak emptying rate (APERi) (46 mL/s/m [95%-range, 29-69] versus 75 mL/s/m [95%-range, 45-178], P < 0.001) were found for patients with myocardial iron overload (R2* > 40 s ) compared with patients with normal myocardial iron levels (R2* < 40 s ). Receiver operating characteristics (ROC) analysis revealed higher potential to indicate myocardial iron overload for the AAEF (area under the ROC curve [AUC] = 0.84; P < 0.0001), APERi (AUC = 0.87; P < 0.0001), and AAEVi (AUC = 0.80; P < 0.0001) compared with LA ejection fraction (LAEF) (AUC = 0.68; P = 0.02) with equal sensitivities and specificities of 82% (AAEF), 79% (APERi), 73% (AAEVi), and 57% (LAEF).

Conclusion: MR parameters of active LA contractile function were associated with myocardial iron overload. This cross-sectional study suggests impaired active LA contractile function to be sensitive to myocardial iron toxicity.

Level Of Evidence: 3 J. Magn. Reson. Imaging 2017;45:535-541.
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http://dx.doi.org/10.1002/jmri.25396DOI Listing
February 2017

The Prevalence of Sickle Cell Disease and Its Implication for Newborn Screening in Germany (Hamburg Metropolitan Area).

Pediatr Blood Cancer 2016 Jan 14;63(1):168-70. Epub 2015 Aug 14.

Bernhard-Nocht-Institute for Tropical Medicine, Hamburg, Germany.

Sickle cell disease is among hereditary diseases with evidence that early diagnoses and treatment improves the clinical outcome. So far sickle cell disease has not been included in the German newborn screening program despite immigration from countries with populations at risk. To determine the birth prevalence we tested 17,018 newborns. High pressure liquid chromatography and subsequent molecular-genetic testing were used for the detection and confirmation of hemoglobin variants. The frequency of sickle cell disease-consistent genotypes was one in 2,385 newborns. Duffy-blood group typing showed evidence that affected children were likely of Sub-Saharan ancestry. An inclusion of sickle cell disease into the German newborn screening seems reasonable.
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http://dx.doi.org/10.1002/pbc.25706DOI Listing
January 2016

Brain iron quantification by MRI in mitochondrial membrane protein-associated neurodegeneration under iron-chelating therapy.

Ann Clin Transl Neurol 2014 Dec 2;1(12):1041-6. Epub 2014 Dec 2.

Department of Diagnostic and Interventional Neuroradiology, University Medical Center Hamburg-Eppendorf Hamburg, Germany.

Therapeutic trials for Neurodegeneration with Brain Iron Accumulation have aimed at a reduction of cerebral iron content. A 13-year-old girl with mitochondrial membrane protein-associated neurodegeneration treated with an iron-chelating agent was monitored by R2 relaxometry, R2* relaxometry, and quantitative susceptibility mapping to estimate the brain iron content. The highly increased brain iron content slowly decreased in the substantia nigra but remained stable for globus pallidus. The estimated iron content was higher by R2* compared to R2 and quantitative susceptibility mapping, a finding not previously observed in the brain of healthy volunteers. A hypothesis explaining this discrepancy is offered.
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http://dx.doi.org/10.1002/acn3.116DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4284129PMC
December 2014

Pancreatic iron and fat assessment by MRI-R2* in patients with iron overload diseases.

J Magn Reson Imaging 2015 Jul 19;42(1):196-203. Epub 2014 Sep 19.

Department of Diagnostic and Interventional Radiology, University Medical Center Hamburg-Eppendorf, Germany.

Background: To determine the pancreatic iron (R2*) and fat content (FC) in comparison to hepatic and cardiac R2* in patients with iron overload disorders like β-thalassemia major (TM), Diamond-Blackfan anemia (DBA) or hereditary hemochromatosis.

Methods: R2* rates were assessed in the liver, heart and pancreas of 42 patients with TM, 29 subjects with other iron overload diseases, and 10 controls using an ECG-gated breathhold sequence (12 echo time [TE] = 1.3-25.7 ms, readout repetition time [TR] = 244 ms). Pancreatic R2* and FC were assessed from TE dependent region of interest based signal intensities performing water-fat chemical shift relaxometry and were compared with laboratory parameters (glucose, HbA1c, amylase and lipase).

Results: A pancreatic iron gradient from tail (R2* = 122 s(-1) ) to head (R2* = 114 s(-1) , P < 10(-4) ) was found. The close association between cardiac and pancreatic R2* was also confirmed in patients with TM and other iron overload diseases (rs  = 0.64, P < 10(-4) ). Receiver operator characteristic analysis (area: 0.89, P < 10(-4) ) identified patients with elevated cardiac iron at a pancreatic R2* cut-off level of 131s(-1) (sensitivity = specificity at 81%). Highest pancreatic R2* (211s(-1) ) and FC (36%) were found in the tail region of diabetic patients with TM.

Conclusion: Pancreatic tail showed highest R2* rates and fat contents, especially in patients with thalassemia. Besides iron accumulation fatty degeneration might be an additional risk factor for the development of diabetes in β-thalassemia major, but this hypothesis needs further studies in prediabetic patients.
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http://dx.doi.org/10.1002/jmri.24752DOI Listing
July 2015

Mechanisms of plasma non-transferrin bound iron generation: insights from comparing transfused diamond blackfan anaemia with sickle cell and thalassaemia patients.

Br J Haematol 2014 Dec 11;167(5):692-6. Epub 2014 Sep 11.

Department of Haematology, University College London, London, UK.

In transfusional iron overload, extra-hepatic iron distribution differs, depending on the underlying condition. Relative mechanisms of plasma non-transferrin bound iron (NTBI) generation may account for these differences. Markers of iron metabolism (plasma NTBI, labile iron, hepcidin, transferrin, monocyte SLC40A1 [ferroportin]), erythropoiesis (growth differentiation factor 15, soluble transferrin receptor) and tissue hypoxia (erythropoietin) were compared in patients with Thalassaemia Major (TM), Sickle Cell Disease and Diamond-Blackfan Anaemia (DBA), with matched transfusion histories. The most striking differences between these conditions were relationships of NTBI to erythropoietic markers, leading us to propose three mechanisms of NTBI generation: iron overload (all), ineffective erythropoiesis (predominantly TM) and low transferrin-iron utilization (DBA).
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http://dx.doi.org/10.1111/bjh.13081DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4577015PMC
December 2014

Administration of recombinant erythropoietin alone does not improve the phenotype in iron refractory iron deficiency anemia patients.

Ann Hematol 2013 Mar 20;92(3):387-94. Epub 2012 Nov 20.

Department of Pediatric Hematology and Oncology, University Medical Center Eppendorf, Martinistrasse 52, 20246, Hamburg, Germany.

Mutations in transmembrane protease, serine 6 (TMPRSS6) cause iron refractory iron deficiency anemia (IRIDA). Parenteral iron administration may slightly improve hemoglobin level but is troublesome for patients. Optimal treatment has yet to be determined. We identified five patients from four independent families displaying the IRIDA picture with truncating biallelic mutations in TMPRSS6, one of which is novel. Liver iron determined by superconducting quantum interference device biosusceptometry ranged from 390 to 720 µg Fe/g wet weight (normal range 100-500; n = 3). Intestinal iron absorption (12 and 32 %, normal range 10-50; n = 2) and 59Fe erythrocyte incorporation after ingestion of 59Fe (57 and 38 %, normal range 70-90; n = 2) were inadequately low for iron-deficient anemic individuals. Baseline serum erythropoietin was elevated or borderline high in four patients. Administration of recombinant human erythropoietin (rhEPO) at up to 273 and 188 U/kg body weight/week alone did not improve anemia or result in a decrease of urinary hepcidin in two individuals. In conclusion, the ability of exogenous rhEPO to increase hemoglobin level appears to be impaired in IRIDA.
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http://dx.doi.org/10.1007/s00277-012-1618-8DOI Listing
March 2013

Pancreatic exocrine function and cardiac iron in patients with iron overload and with thalassemia.

Pediatr Blood Cancer 2011 Oct 10;57(4):674-6. Epub 2011 Jun 10.

University Medical Center Hamburg-Eppendorf, Hamburg, Germany.

Patients with β-thalassemia major at risk of cardiac iron overload have to be identified to undergo myocardial iron measurements by magnetic resonance imaging (MRI), especially, in areas and centers with restricted access to MRI. Measurements of heart iron, liver iron, and pancreatic exocrine function were performed in 44 patients by MRI-R2* [the transverse relaxation rate R2* (= 1/T2*) characterizes the magnetic resonance decay from protons not being in phase with each other in contrast to R2 (= 1/T2)], biomagnetic liver susceptometry (LIC), and pancreatic serum amylase (PAM) and lipase (LIP), respectively. ROC analysis (area: 0.88) for detecting patients with cardiac R2* > 50 sec(-1) (T2* < 20 msec) by LIP revealed a cut-off level of 19 U/L. In conclusion, patients at risk of elevated cardiac iron levels could be identified by the exocrine pancreatic lipase and amylase function parameters.
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http://dx.doi.org/10.1002/pbc.22990DOI Listing
October 2011

Distribution of cardiac iron measured by magnetic resonance imaging (MRI)-R*2.

J Magn Reson Imaging 2010 Nov;32(5):1104-9

University Medical Center Hamburg-Eppendorf, Hamburg, Germany.

Purpose: To assess regional iron distribution by magnetic resonance imaging (MRI)-R(2)* within the heart of patients with β-thalassemia major (TM) and other iron overload diseases.

Materials And Methods: Breathhold electrocardiogram (ECG)-gated MRI (1.5 T) of the heart was used for the measurement of transverse relaxation rates R(2)* in 32 patients (11-79 years). In a mid-papillary short-axis slice divided into septal, anterior, lateral, and posterior quadrants, R(2)* was analyzed from region of interest (ROI)-based signal intensities from 12 echo times (TE = 1.3-26 msec). Typical boundary effects were evaluated in detail.

Results: The segmentation of the cardiac wall resulted in highly significant correlations of R(2)* between septal and all other quadrants. In the patient group with R(2)* < 50 s(-1) (normal), all quadrants show higher normalized median rates (126%-174%) than the septum (P < 10(-4)), while this was relatively smaller in the group with septal R(2)* > 50 s(-1). Typical boundary effects on segmental R(2)* from blood, lung tissue, epicardial fat, and hepatic iron could not be easily separated from segmental iron distribution.

Conclusion: The measurement of MRI-R2* in the interventricular septum is the least affected method by boundary effects to detect patients with iron overload at risk of developing heart failure.
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http://dx.doi.org/10.1002/jmri.22364DOI Listing
November 2010

Erythrocytapheresis: Do Not Forget a Useful Therapy!

Transfus Med Hemother 2008 15;35(1):24-30. Epub 2008 Jan 15.

Department of Transfusion Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.

SUMMARY: In patients with pathologically altered erythrocytes, red blood cell exchange is a very efficient therapeutic measure without important side effects. With increasing migration more patients with e.g. severe malaria or sickle cell anemia have to be treated. In minor or bidirectional ABO-mismatched stem cell transplantations after reduced intensity conditioning, hemolysis can be prevented by prophylactic erythrocytapheresis. Other rare indications for red blood cell exchange are advanced erythropoietic protoporphyria and babesiosis. Sickle cell anemia can be treated with hydroxyurea. Transfusions are administered when necessary, but this results in iron overload in the long term. An expensive but safe and very efficient treatment alternative is red blood cell exchange. In cases with stroke, acute chest syndrome and other severe complications, erythrocytapheresis reproducibly breaks the vicious circle of sickling and increasing oxygen deficiency. At the same time one can aim at an exact end hematocrit. In severe malaria, erythrocytapheresis both reduces parasite load to the designated extent and reconstitutes reduced oxygen transport capacity without serious adverse effects. Here we describe our experience of erythrocytapheresis in long-term prophylaxis of complications in sickle cell anemia and sickle cell thalassemia patients. The documentation of improved iron balance was carried out by liver susceptometry.
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http://dx.doi.org/10.1159/000112044DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3083277PMC
January 2008

Non-transferrin-bound iron during blood transfusion cycles in beta-thalassemia major.

Ann N Y Acad Sci 2005 ;1054:429-32

University Medical Center Hamburg-Eppendorf, Department of Pediatric Hematology/Oncology, Martinistr. 52, Hamburg D-20246, Germany.

Serum non-transferrin-bound iron (NTBI) levels assessed at arbitrary time points during transfusion cycles may not be representative if NTBI is undergoing significant changes during transfusion cycles. In 15 patients with beta-thalassemia major (age: 21 +/- 6 years, liver iron concentration: 2200 +/- 1200 microg/g-liver), NTBI and other hematologic parameters (transferrin saturation, transferrin receptor) were measured weekly. The largest variation of NTBI levels between individual patients was observed at midcycle. For long-term monitoring of NTBI levels, a particular time point relative to the last blood transfusion should be selected for blood drawing.
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http://dx.doi.org/10.1196/annals.1345.074DOI Listing
July 2006
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