Current German Guidelines on Diagnosis and Treatment of Secondary Hemochromatosis in Patients with Congenital Anemias

Abstract

Adequate diagnosis and treatment of secondary hemochromatosis in patients with congenital anemias is important for reducing long-term mortality and morbidity as for improving patients' quality of life. Due to the strong migration movements during recent years, the number of patients in Germany suffering from hemoglobinopathies as some of the most relevant disorders in the context of iron overload (IOL) has increased enormously. Many of these patients had received inadequate medical care in their countries of origin prior to migration, including diagnosis and treatment of IOL. In parallel, various medical developments and achievements took place, including the expansion of stem cell transplant as curative therapeutic option and the introduction of gene therapy for patients with hemoglobinopathies. Diagnostic tools to assess both liver and heart IOL became available at more sites in Germany. Overall experience with iron elimination therapy either as monotherapy or as combination of different chelators increased. All these aspects were considered during revision of the consensus-based guideline on the diagnosis and treatment of secondary IOL in patients with congenital anemias (AWMF Reg. No. 025/029). Here, we briefly summarize the procedure for the revision of the guideline, provide a brief overview of innovations as compared to the previous version dated from 2015, and finally present the consensus recommendations adopted in the current version.

Noninvasive liver iron quantification by MRI using refocused gradient-echo (bSSFP): preliminary results

“…Correlation parameters of this multiple linear correlation were used to derive a relationship to determine the LIC from bSSFP data. The sensitivity and specificity of these bSSFP-LIC values with respect to the reference method were studied for an LIC threshold of 80 µmol/g (4.5 mg/g) relevant for iron chelation therapy [5].…”

Volumetric Evaluation of 3D Multi-Gradient-Echo MRI Data to Assess Whole Liver Iron Distribution by Segmental R2* Analysis: First Experience

“…In addition, we studied whether nonuniform R 2 * distribution depended on R 2 * levels by dividing our participants into two groups according to their average hepatic R 2 * value, group A with R 2 * < 140 s –1 , group B with R 2 * > 140 s –1 , a threshold which corresponds to the LIC threshold for therapy indication of 4.5 mg/g and a recently published R 2 * calibration for the sequence used 5 10 . The significance of differences between the two groups was calculated using the Mann-Whitney-U-Test.…”