Differential diagnosis between AVWS and VWD
| Aspect . | In favor of AVWS . | In favor of VWD . | Limitations . |
|---|---|---|---|
| Personal history | Late onset of bleeding Uneventful surgery before onset of bleeding | Early onset of bleeding No uneventful surgery or no previous high-risk situations | Variable penetrance of VWD |
| Family history | Negative | Positive | Variable penetrance of VWD |
| AVWS-associated disorder | Present | Absent | Coincidental presence of highly prevalent disorders (eg, MGUS in the elderly) |
| Laboratory evaluation | Presence of inhibitor or VWF-binding antibodies | VWF gene mutation | Low frequency of detectable inhibitors in AVWS Alloantibodies in rare cases of VWD type 3 |
| Treatment response | Remission after treatment of underlying disorder Response to IVIG (in IgG-MGUS-associated AVWS) Short-lived response to VWF-containing concentrates or desmopressin | Normal recovery and half-life of VWF-containing concentrate Sustained response to desmopressin | Cannot be assessed before treatment |
| Aspect . | In favor of AVWS . | In favor of VWD . | Limitations . |
|---|---|---|---|
| Personal history | Late onset of bleeding Uneventful surgery before onset of bleeding | Early onset of bleeding No uneventful surgery or no previous high-risk situations | Variable penetrance of VWD |
| Family history | Negative | Positive | Variable penetrance of VWD |
| AVWS-associated disorder | Present | Absent | Coincidental presence of highly prevalent disorders (eg, MGUS in the elderly) |
| Laboratory evaluation | Presence of inhibitor or VWF-binding antibodies | VWF gene mutation | Low frequency of detectable inhibitors in AVWS Alloantibodies in rare cases of VWD type 3 |
| Treatment response | Remission after treatment of underlying disorder Response to IVIG (in IgG-MGUS-associated AVWS) Short-lived response to VWF-containing concentrates or desmopressin | Normal recovery and half-life of VWF-containing concentrate Sustained response to desmopressin | Cannot be assessed before treatment |
VWD indicates von Willebrand disease; VWF, von Willebrand factor; MGUS, monoclonal gammopathy of undetermined significance; and IVIG, intravenous immunoglobulin.