Therapeutic options according to underlying disorder
| Underlying disorder . | Causal treatment . | Additional treatment options . |
|---|---|---|
| Autoimmune disorders | ||
| Systemic lupus erythematosus | Steroids, cyclophosphamide | IVIG (only IgG-MGUS or anti-VWF IgG), plasmapheresis, antifibrinolytics, VWF-containing concentrate, rFVIIa |
| Lymphoproliferative disorders | ||
| MGUS | Usually untreated | |
| Lymphoma, multiple myeloma | Chemotherapy according to entity | |
| Cardiovascular | ||
| Aortic valve stenosis and other anomalies with increased shear stress | Corrective surgery | VWF-containing concentrate, antifibrinolytics |
| Dysfunctional heart valve prosthesis, LVAD | Corrective surgery if applicable | Reduce or withdraw anticoagulation, VWF-containing concentrate |
| Myeloproliferative neoplasia | ||
| Essential thrombocythemia | Cytoreductive therapy, chemotherapy, or stem cell transplantation in case of progression | |
| Polycythemia vera | Phlebotomy, cytoreductive therapy, chemotherapy, or stem cell transplantation in case of progression | Withdraw aspirin (if applicable), desmopressin, antifibrinolytics, VWF-containing concentrate |
| Chronic myeloid leukemia | Tyrosine kinase inhibitors, stem cell transplantation |
| Underlying disorder . | Causal treatment . | Additional treatment options . |
|---|---|---|
| Autoimmune disorders | ||
| Systemic lupus erythematosus | Steroids, cyclophosphamide | IVIG (only IgG-MGUS or anti-VWF IgG), plasmapheresis, antifibrinolytics, VWF-containing concentrate, rFVIIa |
| Lymphoproliferative disorders | ||
| MGUS | Usually untreated | |
| Lymphoma, multiple myeloma | Chemotherapy according to entity | |
| Cardiovascular | ||
| Aortic valve stenosis and other anomalies with increased shear stress | Corrective surgery | VWF-containing concentrate, antifibrinolytics |
| Dysfunctional heart valve prosthesis, LVAD | Corrective surgery if applicable | Reduce or withdraw anticoagulation, VWF-containing concentrate |
| Myeloproliferative neoplasia | ||
| Essential thrombocythemia | Cytoreductive therapy, chemotherapy, or stem cell transplantation in case of progression | |
| Polycythemia vera | Phlebotomy, cytoreductive therapy, chemotherapy, or stem cell transplantation in case of progression | Withdraw aspirin (if applicable), desmopressin, antifibrinolytics, VWF-containing concentrate |
| Chronic myeloid leukemia | Tyrosine kinase inhibitors, stem cell transplantation |
VWF indicates von Willebrand factor.