Case report: A case of acquired von Willebrand syndrome as onset clinical presentation of systemic lupus erythematosus manifested as epistaxis and pulmonary hemorrhage
BackgroundAcquired von Willebrand syndrome (AVWS) is a less common bleeding disorder, primarily manifested as mild to moderate mucocutaneous bleeding and laboratory tests are similar to hereditary von Willebrand disease (VWD). AVWS is secondary to other diseases, and systemic lupus erythematosus (SL...
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| Format: | Article |
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Frontiers Media S.A.
2022-09-01
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| Series: | Frontiers in Pediatrics |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fped.2022.1013764/full |
| _version_ | 1828105321383460864 |
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| author | Songmi Wang Qun Hu Yaxian Chen Xiufen Hu Ning Tang Ai Zhang Aiguo Liu |
| author_facet | Songmi Wang Qun Hu Yaxian Chen Xiufen Hu Ning Tang Ai Zhang Aiguo Liu |
| author_sort | Songmi Wang |
| collection | DOAJ |
| description | BackgroundAcquired von Willebrand syndrome (AVWS) is a less common bleeding disorder, primarily manifested as mild to moderate mucocutaneous bleeding and laboratory tests are similar to hereditary von Willebrand disease (VWD). AVWS is secondary to other diseases, and systemic lupus erythematosus (SLE) is a relatively rare cause.Case presentationWe report a case of AVWS as onset clinical presentation of SLE manifested as epistaxis and pulmonary hemorrhage. A 13-year-old male child presented to the hospital with a six-month history of recurrent epistaxis and a one-month history of anemia. Routine blood tests demonstrated severe normocytic anemia and normal platelet count. Von Willebrand test revealed a significantly lower level. High-resolution chest computed tomography (CT) showed patchy ground glass opacities consistent with hemorrhagic changes. After ruling out the family history, the patient was diagnosed with AVWS. Additional tests confirmed positive antinuclear and anti-Sm antibodies. The underlying SLE was diagnosed and treated with methylprednisolone with disease recovery.ConclusionWe recommend screening for bleeding disorders in patients with recurrent epistaxis. AVWS should be considered when laboratory findings suggest hereditary von Willebrand disease without a personal or familial history of bleeding. In addition, the underlying disease should be explored. |
| first_indexed | 2024-04-11T10:01:24Z |
| format | Article |
| id | doaj.art-14d3c7e86f94427ead66bba45e87de9b |
| institution | Directory Open Access Journal |
| issn | 2296-2360 |
| language | English |
| last_indexed | 2024-04-11T10:01:24Z |
| publishDate | 2022-09-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Pediatrics |
| spelling | doaj.art-14d3c7e86f94427ead66bba45e87de9b2022-12-22T04:30:25ZengFrontiers Media S.A.Frontiers in Pediatrics2296-23602022-09-011010.3389/fped.2022.10137641013764Case report: A case of acquired von Willebrand syndrome as onset clinical presentation of systemic lupus erythematosus manifested as epistaxis and pulmonary hemorrhageSongmi Wang0Qun Hu1Yaxian Chen2Xiufen Hu3Ning Tang4Ai Zhang5Aiguo Liu6Department of Pediatrics, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, ChinaDepartment of Pediatrics, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, ChinaDepartment of Pediatrics, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, ChinaDepartment of Pediatrics, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, ChinaDepartment of Clinical Laboratory, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, ChinaDepartment of Pediatrics, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, ChinaDepartment of Pediatrics, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, ChinaBackgroundAcquired von Willebrand syndrome (AVWS) is a less common bleeding disorder, primarily manifested as mild to moderate mucocutaneous bleeding and laboratory tests are similar to hereditary von Willebrand disease (VWD). AVWS is secondary to other diseases, and systemic lupus erythematosus (SLE) is a relatively rare cause.Case presentationWe report a case of AVWS as onset clinical presentation of SLE manifested as epistaxis and pulmonary hemorrhage. A 13-year-old male child presented to the hospital with a six-month history of recurrent epistaxis and a one-month history of anemia. Routine blood tests demonstrated severe normocytic anemia and normal platelet count. Von Willebrand test revealed a significantly lower level. High-resolution chest computed tomography (CT) showed patchy ground glass opacities consistent with hemorrhagic changes. After ruling out the family history, the patient was diagnosed with AVWS. Additional tests confirmed positive antinuclear and anti-Sm antibodies. The underlying SLE was diagnosed and treated with methylprednisolone with disease recovery.ConclusionWe recommend screening for bleeding disorders in patients with recurrent epistaxis. AVWS should be considered when laboratory findings suggest hereditary von Willebrand disease without a personal or familial history of bleeding. In addition, the underlying disease should be explored.https://www.frontiersin.org/articles/10.3389/fped.2022.1013764/fullepistaxispulmonary hemorrhagepediatricsacquired von Willebrand syndromesystemic lupus erythematosus |
| spellingShingle | Songmi Wang Qun Hu Yaxian Chen Xiufen Hu Ning Tang Ai Zhang Aiguo Liu Case report: A case of acquired von Willebrand syndrome as onset clinical presentation of systemic lupus erythematosus manifested as epistaxis and pulmonary hemorrhage Frontiers in Pediatrics epistaxis pulmonary hemorrhage pediatrics acquired von Willebrand syndrome systemic lupus erythematosus |
| title | Case report: A case of acquired von Willebrand syndrome as onset clinical presentation of systemic lupus erythematosus manifested as epistaxis and pulmonary hemorrhage |
| title_full | Case report: A case of acquired von Willebrand syndrome as onset clinical presentation of systemic lupus erythematosus manifested as epistaxis and pulmonary hemorrhage |
| title_fullStr | Case report: A case of acquired von Willebrand syndrome as onset clinical presentation of systemic lupus erythematosus manifested as epistaxis and pulmonary hemorrhage |
| title_full_unstemmed | Case report: A case of acquired von Willebrand syndrome as onset clinical presentation of systemic lupus erythematosus manifested as epistaxis and pulmonary hemorrhage |
| title_short | Case report: A case of acquired von Willebrand syndrome as onset clinical presentation of systemic lupus erythematosus manifested as epistaxis and pulmonary hemorrhage |
| title_sort | case report a case of acquired von willebrand syndrome as onset clinical presentation of systemic lupus erythematosus manifested as epistaxis and pulmonary hemorrhage |
| topic | epistaxis pulmonary hemorrhage pediatrics acquired von Willebrand syndrome systemic lupus erythematosus |
| url | https://www.frontiersin.org/articles/10.3389/fped.2022.1013764/full |
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