Suggested AVWS management strategies
| . | Treatment . | Notes . |
|---|---|---|
| Management of acute bleeding | ||
| DDAVP | Rapid fall-off in plasma VWF response may be seen | |
| Frequent monitoring of VWF, including a functional assay (eg, VWF:RCo) | ||
| May not be suitable in elderly patients and those with history of cardiovascular disease | ||
| VWF-containing concentrate | Rapid fall-off in plasma VWF response may be seen | |
| Frequent monitoring of VWF, including a functional assay (eg, VWF:RCo) | ||
| Intravenous immunoglobulin (IVIG) | Patients with IgG paraprotein often respond well | |
| Increase in plasma VWF typically observed after 12-72 h | ||
| Duration of effect may vary from few days up to 3 wk | ||
| Patients with IgM paraprotein unlikely to respond | ||
| Plasmapheresis | Short duration of response; bridge to definitive treatment | |
| Antifibrinolytic therapy | May be useful to treat minor bleeds, or as an adjunctive therapy for more serious hemorrhage | |
| Recombinant factor VIIa | Few reports of use in AVWS | |
| Caution regarding risk of thromboembolism, especially in elderly patients | ||
| Treatment of underlying disorder | ||
| Cardiovascular disorders | Surgical repair | |
| Myeloproliferative disorders | Cytoreductive therapy | |
| Lymphoproliferative/myeloma/malignancy | Chemotherapy | |
| Monoclonal gammopathy of undetermined significance | Usually untreated | |
| Autoimmune disorders | Immunosuppression | |
| Hypothyroidism | Thyroxine replacement |
| . | Treatment . | Notes . |
|---|---|---|
| Management of acute bleeding | ||
| DDAVP | Rapid fall-off in plasma VWF response may be seen | |
| Frequent monitoring of VWF, including a functional assay (eg, VWF:RCo) | ||
| May not be suitable in elderly patients and those with history of cardiovascular disease | ||
| VWF-containing concentrate | Rapid fall-off in plasma VWF response may be seen | |
| Frequent monitoring of VWF, including a functional assay (eg, VWF:RCo) | ||
| Intravenous immunoglobulin (IVIG) | Patients with IgG paraprotein often respond well | |
| Increase in plasma VWF typically observed after 12-72 h | ||
| Duration of effect may vary from few days up to 3 wk | ||
| Patients with IgM paraprotein unlikely to respond | ||
| Plasmapheresis | Short duration of response; bridge to definitive treatment | |
| Antifibrinolytic therapy | May be useful to treat minor bleeds, or as an adjunctive therapy for more serious hemorrhage | |
| Recombinant factor VIIa | Few reports of use in AVWS | |
| Caution regarding risk of thromboembolism, especially in elderly patients | ||
| Treatment of underlying disorder | ||
| Cardiovascular disorders | Surgical repair | |
| Myeloproliferative disorders | Cytoreductive therapy | |
| Lymphoproliferative/myeloma/malignancy | Chemotherapy | |
| Monoclonal gammopathy of undetermined significance | Usually untreated | |
| Autoimmune disorders | Immunosuppression | |
| Hypothyroidism | Thyroxine replacement |