Venous Thrombosis in Acquired Hemophilia: The Complex Management of Competing Pathologies
Introduction Venous thrombosis is rare in the setting of factor VIII (FVIII) deficiency. Cases of deep vein thrombosis (DVT) have been described in hemophiliacs after recent major surgery, or in association with the administration of FVIII concentrate and activated prothrombin complex concentrates,...
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| Format: | Article |
| Language: | English |
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Georg Thieme Verlag KG
2019-10-01
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| Series: | TH Open |
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| Online Access: | http://www.thieme-connect.de/DOI/DOI?10.1055/s-0039-1698414 |
| _version_ | 1819088372915765248 |
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| author | Manu Chhabra Zhen Wan Stephanie Hii Joseph Rajendran Kuperan Ponnudurai Bingwen Eugene Fan |
| author_facet | Manu Chhabra Zhen Wan Stephanie Hii Joseph Rajendran Kuperan Ponnudurai Bingwen Eugene Fan |
| author_sort | Manu Chhabra |
| collection | DOAJ |
| description | Introduction Venous thrombosis is rare in the setting of factor VIII (FVIII) deficiency. Cases of deep vein thrombosis (DVT) have been described in hemophiliacs after recent major surgery, or in association with the administration of FVIII concentrate and activated prothrombin complex concentrates, but occurrence of spontaneous DVT is even more uncommon.
Aim We describe the challenging management of extensive DVT in a patient with acquired hemophilia A with concurrent hemorrhagic manifestations and review similar published cases.
Methods We summarize a series of 10 cases with the following demographics: 6 males and 4 females; median age at presentation of 65 (21–80); mean inhibitor titer of 68.5 Bethesda Units (BU 1.9 to BU 350).
Results Four cases were idiopathic and six had associated conditions (cancer [two cases], recent pregnancy [two cases], and recent surgery [two cases]). Three cases had an inferior vena cava filter inserted for acute lower limb DVT/pulmonary embolism. Inhibitor eradication was achieved with high-dose steroids with or without cyclophosphamide, and adjunct Rituximab administration was used in three cases. One patient received concurrent therapeutic plasma exchange (TPE). Inhibitor eradication was fastest with concurrent TPE at 6 days (range: 6–733 days). The 30-day survival was 90%.
Conclusions There was adequate response of inhibitors to immunosuppression with steroids and cyclophosphamide therapy. For more refractory disease, Rituximab is emerging as a beneficial and cost-effective adjunct with better rates of complete remission, and the threshold for its use may be lowered in this complex cohort with dual competing pathologies. |
| first_indexed | 2024-12-21T21:51:00Z |
| format | Article |
| id | doaj.art-4b27cde9df27484081cc39ecaf4b476f |
| institution | Directory Open Access Journal |
| issn | 2512-9465 2512-9465 |
| language | English |
| last_indexed | 2024-12-21T21:51:00Z |
| publishDate | 2019-10-01 |
| publisher | Georg Thieme Verlag KG |
| record_format | Article |
| series | TH Open |
| spelling | doaj.art-4b27cde9df27484081cc39ecaf4b476f2022-12-21T18:49:05ZengGeorg Thieme Verlag KGTH Open2512-94652512-94652019-10-010304e325e33010.1055/s-0039-1698414Venous Thrombosis in Acquired Hemophilia: The Complex Management of Competing PathologiesManu Chhabra0Zhen Wan Stephanie Hii1Joseph Rajendran2Kuperan Ponnudurai3Bingwen Eugene Fan4Department of Hematology, Tan Tock Seng Hospital, Singapore, SingaporeKhoo Teck Puat—National University Children's Medical Institute, National University Health System, Singapore, SingaporeDepartment of Hematology, Tan Tock Seng Hospital, Singapore, SingaporeDepartment of Hematology, Tan Tock Seng Hospital, Singapore, SingaporeDepartment of Hematology, Tan Tock Seng Hospital, Singapore, SingaporeIntroduction Venous thrombosis is rare in the setting of factor VIII (FVIII) deficiency. Cases of deep vein thrombosis (DVT) have been described in hemophiliacs after recent major surgery, or in association with the administration of FVIII concentrate and activated prothrombin complex concentrates, but occurrence of spontaneous DVT is even more uncommon. Aim We describe the challenging management of extensive DVT in a patient with acquired hemophilia A with concurrent hemorrhagic manifestations and review similar published cases. Methods We summarize a series of 10 cases with the following demographics: 6 males and 4 females; median age at presentation of 65 (21–80); mean inhibitor titer of 68.5 Bethesda Units (BU 1.9 to BU 350). Results Four cases were idiopathic and six had associated conditions (cancer [two cases], recent pregnancy [two cases], and recent surgery [two cases]). Three cases had an inferior vena cava filter inserted for acute lower limb DVT/pulmonary embolism. Inhibitor eradication was achieved with high-dose steroids with or without cyclophosphamide, and adjunct Rituximab administration was used in three cases. One patient received concurrent therapeutic plasma exchange (TPE). Inhibitor eradication was fastest with concurrent TPE at 6 days (range: 6–733 days). The 30-day survival was 90%. Conclusions There was adequate response of inhibitors to immunosuppression with steroids and cyclophosphamide therapy. For more refractory disease, Rituximab is emerging as a beneficial and cost-effective adjunct with better rates of complete remission, and the threshold for its use may be lowered in this complex cohort with dual competing pathologies.http://www.thieme-connect.de/DOI/DOI?10.1055/s-0039-1698414acquired hemophiliadeep vein thrombosisthromboembolism |
| spellingShingle | Manu Chhabra Zhen Wan Stephanie Hii Joseph Rajendran Kuperan Ponnudurai Bingwen Eugene Fan Venous Thrombosis in Acquired Hemophilia: The Complex Management of Competing Pathologies TH Open acquired hemophilia deep vein thrombosis thromboembolism |
| title | Venous Thrombosis in Acquired Hemophilia: The Complex Management of Competing Pathologies |
| title_full | Venous Thrombosis in Acquired Hemophilia: The Complex Management of Competing Pathologies |
| title_fullStr | Venous Thrombosis in Acquired Hemophilia: The Complex Management of Competing Pathologies |
| title_full_unstemmed | Venous Thrombosis in Acquired Hemophilia: The Complex Management of Competing Pathologies |
| title_short | Venous Thrombosis in Acquired Hemophilia: The Complex Management of Competing Pathologies |
| title_sort | venous thrombosis in acquired hemophilia the complex management of competing pathologies |
| topic | acquired hemophilia deep vein thrombosis thromboembolism |
| url | http://www.thieme-connect.de/DOI/DOI?10.1055/s-0039-1698414 |
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