Questions 498-499: Specify inherited abnormalities of metabolism classification:
Indicate the inherited abnormalities of metabolism disease classification at diagnosis. If the subtype is not listed, report as “other inherited metabolic disorder” and specify the reported disease in question 499. If a certain disease becomes a common indication for HCT, the CIBMTR will add the disease as a separate category.
Question 500: Report the Loes composite score (Adrenoleukodystrophy (ALD) only):
The Loes composite score is often used to assess disease/progression for recipients with ALD. The Loes composite score is a rating from 0-34, this signifies the severity of abnormalities detected in the brain after evaluation of MRI. Report the Loes composite score in question 500, if the score is unknown, check with a transplant physician to determine this value.
|Question Number||Date of Change||Add/Remove/Modify||Description||Reasoning (If applicable)|
|Q498 – 499||10/29/2021||Add||The hereditary diffuse leukoencephalopathy red warning box was added above Q498 – 499: Hereditary diffuse leukoencephalopathy (HDLS): If the primary disease for infusion is HDLS, select Other Inherited Metabolic Disorder as the classification and specify ‘hereditary diffuse leukoencephalopathy (HDLS).’ In addition, all recipients whose primary disease is reported as a ‘leukodystrophy’ (listed under Inherited Disorders of Metabolism) are required to complete Pre-Infusion Leukodystrophies (2037) and Post-Infusion Leukodystrophies (2137) forms. For recipients where the classification is ‘hereditary diffuse leukoencephalopathy (HDLS),’ submit a ticket through CIBMTR Customer Support to request the Pre-Infusion Leukodystrophies (2037) and Post-Infusion Leukodystrophies (2137) forms come due. With the Summer 2022 release, the Disease Classification (2402) form will be updated with an explicit option for this disease and the 2037 / 2137 forms will automatically come due.||Due to the addition of the 2037 / 2137 (version 1) forms. HDLS is an option on the 2037 / 2137 forms; however, the 2402 has not yet been revised|
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