| Autor: |
Shah, Farrukh T., Porter, John B., Sadasivam, Nandini, Kaya, Banu, Moon, James C., Velangi, Mark, Ako, Emmanuel, Pancham, Shivan |
| Předmět: |
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| Zdroj: |
British Journal of Haematology; Jan2022, Vol. 196 Issue 2, p336-350, 15p |
| Abstrakt: |
There may also be considerable inter-centre variability even if the same methodology is being used to acquire the data.59 Transfusion-dependent patients should be having tailored MRI assessments of LIC routinely with a frequency dependent on the severity of iron burden, the intensity of chelation and the concordance with iron chelation therapy60,61 Cardiac iron Cardiac T2* is the current standard measure for assessing myocardial iron deposition and T1 mapping is being used in research settings. Keywords: diamond-blackfan; thalassaemia; transfusion; chelation EN diamond-blackfan thalassaemia transfusion chelation 336 350 15 01/17/22 20220115 NES 220115 Methodology This guideline was compiled according to the BSH process at https://b-s-h.org.uk/guidelines/proposing-and-writing-a-new-bsh-guideline/. Patients with transfusion-dependent DBA are more likely to develop severe IOL compared to other transfusion-dependent patients,12,13 with a greater propensity of myocardial IOL and higher NTBI levels.14 Some CDA-1 patients may have concurrent therapy with interferon to limit blood transfusion. NTRIA patients should be assessed on a disease and individual basis and chelation therapy or venesection (if Hb is adequate) considered if there is evidence of IOL (ferritin > 500 g/l or LIC > 5mg/g/dry weight) (2C).; SCD patients receiving top-up transfusions should be commenced on iron chelation as per TDT patients (1B). [Extracted from the article] |
| Databáze: |
Complementary Index |
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