Budd-Chiari syndrome as the presenting feature of systemic immunoglobulin light chain amyloidosis
Budd-Chiari syndrome (BCS), characterised by hepatic venous outflow obstruction, is frequently associated with hypercoagulable states. Systemic immunoglobulin light chain (AL) amyloidosis, on the other hand, commonly presents with bleeding complications. The association between the two diseases is n...
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| Format: | Article |
| Language: | English |
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SMC MEDIA SRL
2025-03-01
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| Series: | European Journal of Case Reports in Internal Medicine |
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| Online Access: | https://www.ejcrim.com/index.php/EJCRIM/article/view/5153 |
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| author | João Luís Miranda Catarina Salvado Francisca Carmo Adriana Guedes Albina Moreira Tiago Guimarães Costa Leonor Silva |
| author_facet | João Luís Miranda Catarina Salvado Francisca Carmo Adriana Guedes Albina Moreira Tiago Guimarães Costa Leonor Silva |
| author_sort | João Luís Miranda |
| collection | DOAJ |
| description | Budd-Chiari syndrome (BCS), characterised by hepatic venous outflow obstruction, is frequently associated with hypercoagulable states. Systemic immunoglobulin light chain (AL) amyloidosis, on the other hand, commonly presents with bleeding complications. The association between the two diseases is not common, but some cases have been described in the past. We report a case of a 58-year-old male who presented with lower limb oedema, abdominal distention and severe fatigue, ultimately diagnosed with BCS secondary to systemic AL amyloidosis. This case highlights the rare association between AL amyloidosis and BCS, likely precipitated by a nephrotic syndrome-induced hypercoagulable state, and emphasises the need for clinicians to consider systemic amyloidosis when investigating the aetiology of BCS, even in the absence of typical bleeding manifestations. |
| format | Article |
| id | doaj-art-bb9f3e5e6496462a8641052ceec9976f |
| institution | DOAJ |
| issn | 2284-2594 |
| language | English |
| publishDate | 2025-03-01 |
| publisher | SMC MEDIA SRL |
| record_format | Article |
| series | European Journal of Case Reports in Internal Medicine |
| spelling | doaj-art-bb9f3e5e6496462a8641052ceec9976f2025-08-20T03:06:21ZengSMC MEDIA SRLEuropean Journal of Case Reports in Internal Medicine2284-25942025-03-0110.12890/2025_0051534688Budd-Chiari syndrome as the presenting feature of systemic immunoglobulin light chain amyloidosisJoão Luís Miranda0Catarina Salvado1Francisca Carmo2https://orcid.org/0000-0001-7805-6424Adriana Guedes3Albina Moreira4Tiago Guimarães Costa5Leonor Silva6Serviço de Medicina Interna, Unidade Local de Saúde Gaia e Espinho, Vila Nova de Gaia, PortugalServiço de Medicina Interna, Unidade Local de Saúde Gaia e Espinho, Vila Nova de Gaia, PortugalServiço de Medicina Interna, Unidade Local de Saúde Gaia e Espinho, Vila Nova de Gaia, PortugalServiço de Medicina Interna, Unidade Local de Saúde Gaia e Espinho, Vila Nova de Gaia, PortugalServiço de Medicina Interna, Unidade Local de Saúde Gaia e Espinho, Vila Nova de Gaia, PortugalServiço de Medicina Interna, Unidade Local de Saúde Gaia e Espinho, Vila Nova de Gaia, PortugalServiço de Medicina Interna, Unidade Local de Saúde Gaia e Espinho, Vila Nova de Gaia, PortugalBudd-Chiari syndrome (BCS), characterised by hepatic venous outflow obstruction, is frequently associated with hypercoagulable states. Systemic immunoglobulin light chain (AL) amyloidosis, on the other hand, commonly presents with bleeding complications. The association between the two diseases is not common, but some cases have been described in the past. We report a case of a 58-year-old male who presented with lower limb oedema, abdominal distention and severe fatigue, ultimately diagnosed with BCS secondary to systemic AL amyloidosis. This case highlights the rare association between AL amyloidosis and BCS, likely precipitated by a nephrotic syndrome-induced hypercoagulable state, and emphasises the need for clinicians to consider systemic amyloidosis when investigating the aetiology of BCS, even in the absence of typical bleeding manifestations.https://www.ejcrim.com/index.php/EJCRIM/article/view/5153budd-chiari syndromeal amyloidosisnephrotic syndromeliver failure |
| spellingShingle | João Luís Miranda Catarina Salvado Francisca Carmo Adriana Guedes Albina Moreira Tiago Guimarães Costa Leonor Silva Budd-Chiari syndrome as the presenting feature of systemic immunoglobulin light chain amyloidosis European Journal of Case Reports in Internal Medicine budd-chiari syndrome al amyloidosis nephrotic syndrome liver failure |
| title | Budd-Chiari syndrome as the presenting feature of systemic immunoglobulin light chain amyloidosis |
| title_full | Budd-Chiari syndrome as the presenting feature of systemic immunoglobulin light chain amyloidosis |
| title_fullStr | Budd-Chiari syndrome as the presenting feature of systemic immunoglobulin light chain amyloidosis |
| title_full_unstemmed | Budd-Chiari syndrome as the presenting feature of systemic immunoglobulin light chain amyloidosis |
| title_short | Budd-Chiari syndrome as the presenting feature of systemic immunoglobulin light chain amyloidosis |
| title_sort | budd chiari syndrome as the presenting feature of systemic immunoglobulin light chain amyloidosis |
| topic | budd-chiari syndrome al amyloidosis nephrotic syndrome liver failure |
| url | https://www.ejcrim.com/index.php/EJCRIM/article/view/5153 |
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