loading...| Preferred Name |
AL amyloidosis |
|
| Synonyms |
Light-chain amyloidosis |
|
| Definitions |
A clonal B-cell disorder characterized by the aggregation and deposition of insoluble amyloid fibrils derived from misfolding of monoclonal immunoglobulin light chains. It usually presents as systemic AL amyloidosis with involvement of one or more parenchymal organ(s) and, less frequently, as localized amyloidosis with usually nodular deposits restricted to a single organ and/or system. |
|
| ID |
http://www.orpha.net/ORDO/Orphanet_85443 |
|
| alternative_term |
Light-chain amyloidosis |
|
| definition |
A clonal B-cell disorder characterized by the aggregation and deposition of insoluble amyloid fibrils derived from misfolding of monoclonal immunoglobulin light chains. It usually presents as systemic AL amyloidosis with involvement of one or more parenchymal organ(s) and, less frequently, as localized amyloidosis with usually nodular deposits restricted to a single organ and/or system. |
|
| definition_citation |
Orphanet |
|
| expertlink |
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=en&Expert=85443 |
|
| has_age_of_onset |
Adult |
|
| has_inheritance |
Not applicable |
|
| hasDbXref |
OMIM:254500 MeSH:D000075363 ICD-10:E85.9 ICD-11:5D00.0 UMLS:C0268381 MedDRA:10036673 ICD-10:E85.4 |
|
| label |
AL amyloidosis |
|
| notation |
ORPHA:85443 |
|
| part_of |
http://www.orpha.net/ORDO/Orphanet_209013 http://www.orpha.net/ORDO/Orphanet_69 http://www.orpha.net/ORDO/Orphanet_98282 http://www.orpha.net/ORDO/Orphanet_217598 |
|
| prefixIRI |
ORDO:Orphanet_85443 |
|
| prefLabel |
AL amyloidosis |
|
| present_in |
Netherlands AND has_annual_incidence_average_value : 1.146 AND has_annual_incidence_range : 1-9 / 100 000 Canada AND has_annual_incidence_average_value : 1.076 AND has_annual_incidence_range : 1-9 / 100 000 Lithuania AND has_point_prevalence_average_value : 5.61 AND has_point_prevalence_range : 1-9 / 100 000 Croatia AND has_annual_incidence_average_value : 1.156 AND has_annual_incidence_range : 1-9 / 100 000 Russian Federation AND has_point_prevalence_average_value : 4.569 AND has_point_prevalence_range : 1-9 / 100 000 Slovenia AND has_point_prevalence_average_value : 5.756 AND has_point_prevalence_range : 1-9 / 100 000 Argentina AND has_annual_incidence_average_value : 6.0 AND has_annual_incidence_range : 1-9 / 1 000 000 Worldwide AND has_point_prevalence_average_value : 5.127 AND has_point_prevalence_range : 1-9 / 100 000 Korea, Republic of AND has_annual_incidence_average_value : 0.997 AND has_annual_incidence_range : 1-9 / 1 000 000 Taiwan, Province of China AND has_annual_incidence_average_value : 1.001 AND has_annual_incidence_range : 1-9 / 100 000 Belgium AND has_point_prevalence_average_value : 5.54 AND has_point_prevalence_range : 1-9 / 1 000 000 Hungary AND has_annual_incidence_average_value : 1.088 AND has_annual_incidence_range : 1-9 / 100 000 Belgium AND has_annual_incidence_average_value : 1.122 AND has_annual_incidence_range : 1-9 / 100 000 Germany AND has_point_prevalence_average_value : 6.459 AND has_point_prevalence_range : 1-9 / 100 000 Russian Federation AND has_annual_incidence_average_value : 0.93 AND has_annual_incidence_range : 1-9 / 1 000 000 Slovakia AND has_point_prevalence_average_value : 4.76 AND has_point_prevalence_range : 1-9 / 100 000 Hungary AND has_point_prevalence_average_value : 5.418 AND has_point_prevalence_range : 1-9 / 100 000 France AND has_annual_incidence_average_value : 1.152 AND has_annual_incidence_range : 1-9 / 100 000 France AND has_point_prevalence_average_value : 5.692 AND has_point_prevalence_range : 1-9 / 100 000 Greece AND has_point_prevalence_average_value : 6.219 AND has_point_prevalence_range : 1-9 / 100 000 United Kingdom AND has_annual_incidence_average_value : 1.078 AND has_annual_incidence_range : 1-9 / 100 000 Croatia AND has_point_prevalence_average_value : 5.801 AND has_point_prevalence_range : 1-9 / 100 000 Canada AND has_point_prevalence_average_value : 5.215 AND has_point_prevalence_range : 1-9 / 100 000 Bulgaria AND has_point_prevalence_average_value : 5.892 AND has_point_prevalence_range : 1-9 / 100 000 Romania AND has_annual_incidence_average_value : 1.107 AND has_annual_incidence_range : 1-9 / 100 000 Korea, Republic of AND has_point_prevalence_average_value : 4.822 AND has_point_prevalence_range : 1-9 / 100 000 Japan AND has_annual_incidence_average_value : 1.43 AND has_annual_incidence_range : 1-9 / 100 000 Latvia AND has_point_prevalence_average_value : 5.71 AND has_point_prevalence_range : 1-9 / 100 000 Finland AND has_point_prevalence_average_value : 5.885 AND has_point_prevalence_range : 1-9 / 100 000 Sweden AND has_point_prevalence_average_value : 5.667 AND has_point_prevalence_range : 1-9 / 100 000 Poland AND has_annual_incidence_average_value : 1.044 AND has_annual_incidence_range : 1-9 / 100 000 Brazil AND has_point_prevalence_average_value : 3.222 AND has_point_prevalence_range : 1-9 / 100 000 Czech Republic AND has_point_prevalence_average_value : 5.462 AND has_point_prevalence_range : 1-9 / 100 000 Austria AND has_annual_incidence_average_value : 1.166 AND has_annual_incidence_range : 1-9 / 100 000 Ireland AND has_point_prevalence_average_value : 4.448 AND has_point_prevalence_range : 1-9 / 100 000 Slovenia AND has_annual_incidence_average_value : 1.167 AND has_annual_incidence_range : 1-9 / 100 000 United States AND has_annual_incidence_average_value : 0.991 AND has_annual_incidence_range : 1-9 / 1 000 000 Slovakia AND has_annual_incidence_average_value : 0.968 AND has_annual_incidence_range : 1-9 / 1 000 000 Cyprus AND has_annual_incidence_average_value : 0.894 AND has_annual_incidence_range : 1-9 / 1 000 000 Poland AND has_point_prevalence_average_value : 5.134 AND has_point_prevalence_range : 1-9 / 100 000 Lithuania AND has_annual_incidence_average_value : 1.109 AND has_annual_incidence_range : 1-9 / 100 000 Italy AND has_point_prevalence_average_value : 6.538 AND has_point_prevalence_range : 1-9 / 100 000 Taiwan, Province of China AND has_point_prevalence_average_value : 4.852 AND has_point_prevalence_range : 1-9 / 100 000 Latvia AND has_annual_incidence_average_value : 1.123 AND has_annual_incidence_range : 1-9 / 100 000 Liechtenstein AND has_point_prevalence_average_value : 5.809 AND has_point_prevalence_range : 1-9 / 100 000 Luxembourg AND has_annual_incidence_average_value : 0.968 AND has_annual_incidence_range : 1-9 / 1 000 000 Cyprus AND has_point_prevalence_average_value : 4.299 AND has_point_prevalence_range : 1-9 / 100 000 Europe AND has_annual_incidence_average_value : 1.1177 AND has_annual_incidence_range : 1-9 / 100 000 Sweden AND has_annual_incidence_average_value : 1.141 AND has_annual_incidence_range : 1-9 / 100 000 Romania AND has_point_prevalence_average_value : 5.537 AND has_point_prevalence_range : 1-9 / 100 000 Spain AND has_annual_incidence_average_value : 1.193 AND has_annual_incidence_range : 1-9 / 100 000 Luxembourg AND has_point_prevalence_average_value : 4.653 AND has_point_prevalence_range : 1-9 / 100 000 Europe AND has_point_prevalence_average_value : 5.5311 AND has_point_prevalence_range : 1-9 / 100 000 Malta AND has_annual_incidence_average_value : 1.147 AND has_annual_incidence_range : 1-9 / 100 000 Greece AND has_annual_incidence_average_value : 1.254 AND has_annual_incidence_range : 1-9 / 100 000 Estonia AND has_point_prevalence_average_value : 5.51 AND has_point_prevalence_range : 1-9 / 100 000 Spain AND has_point_prevalence_average_value : 5.864 AND has_point_prevalence_range : 1-9 / 100 000 Denmark AND has_annual_incidence_average_value : 1.124 AND has_annual_incidence_range : 1-9 / 100 000 Netherlands AND has_point_prevalence_average_value : 5.672 AND has_point_prevalence_range : 1-9 / 100 000 Estonia AND has_annual_incidence_average_value : 1.107 AND has_annual_incidence_range : 1-9 / 100 000 Ireland AND has_annual_incidence_average_value : 0.913 AND has_annual_incidence_range : 1-9 / 1 000 000 Iceland AND has_annual_incidence_average_value : 0.948 AND has_annual_incidence_range : 1-9 / 1 000 000 Portugal AND has_point_prevalence_average_value : 6.216 AND has_point_prevalence_range : 1-9 / 100 000 Portugal AND has_annual_incidence_average_value : 1.25 AND has_annual_incidence_range : 1-9 / 100 000 Finland AND has_annual_incidence_average_value : 1.187 AND has_annual_incidence_range : 1-9 / 100 000 Iceland AND has_point_prevalence_average_value : 4.643 AND has_point_prevalence_range : 1-9 / 100 000 Czech Republic AND has_annual_incidence_average_value : 1.109 AND has_annual_incidence_range : 1-9 / 100 000 Malta AND has_point_prevalence_average_value : 5.641 AND has_point_prevalence_range : 1-9 / 100 000 Brazil AND has_annual_incidence_average_value : 0.672 AND has_annual_incidence_range : 1-9 / 1 000 000 Worldwide AND has_annual_incidence_average_value : 1.044 AND has_annual_incidence_range : 1-9 / 100 000 Bulgaria AND has_annual_incidence_average_value : 1.174 AND has_annual_incidence_range : 1-9 / 100 000 Austria AND has_point_prevalence_average_value : 5.77 AND has_point_prevalence_range : 1-9 / 100 000 Japan AND has_point_prevalence_average_value : 7.108 AND has_point_prevalence_range : 1-9 / 100 000 Norway AND has_annual_incidence_average_value : 1.018 AND has_annual_incidence_range : 1-9 / 100 000 Italy AND has_annual_incidence_average_value : 1.329 AND has_annual_incidence_range : 1-9 / 100 000 Denmark AND has_point_prevalence_average_value : 5.568 AND has_point_prevalence_range : 1-9 / 100 000 United Kingdom AND has_point_prevalence_average_value : 5.316 AND has_point_prevalence_range : 1-9 / 100 000 Norway AND has_point_prevalence_average_value : 4.977 AND has_point_prevalence_range : 1-9 / 100 000 Germany AND has_annual_incidence_average_value : 1.294 AND has_annual_incidence_range : 1-9 / 100 000 Liechtenstein AND has_annual_incidence_average_value : 1.174 AND has_annual_incidence_range : 1-9 / 100 000 United States AND has_point_prevalence_average_value : 4.842 AND has_point_prevalence_range : 1-9 / 100 000 |
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| treeView |
http://www.orpha.net/ORDO/Orphanet_209013 http://www.orpha.net/ORDO/Orphanet_69 http://www.orpha.net/ORDO/Orphanet_98282 http://www.orpha.net/ORDO/Orphanet_217598 |
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| subClassOf |