Classification of acquired von Willebrand syndrome.
| Diseases . | Phenotype . | Mechanism(s) . | Effective Tx . | Relative frequency, % . |
|---|---|---|---|---|
| Abbreviations: MGUS, monoclonal gammopathy of undetermined significance; SLE, systemic lupus erythematosus; Tx, treatment | ||||
| Lymphoproliferative diseases | 38 | |||
| MGUS IgG | Type 2 | Autoantibody | IVIg | |
| MGUS IgM | Type 2 | Unknown | None | |
| Myeloproliferative diseases | ||||
| Primary thrombosis | Type 2 | Increased proteolysis | Platelet reduction | 17 |
| Neoplasia | 5 | |||
| Wilm tumor | Type 1–3 | Unknown | Tumor reduction | |
| Immune | 4 | |||
| SLE | Type 2 | Autoantibody | Prednisone, IVIg | |
| Cardiovascular | Mild type 2 | Incrased proteolysis | Surgical repair | 15 |
| Hypothyroidism | Type 1 | Decreased synthesis | Thyroid replacement | 5 |
| Miscellaneous | 16 | |||
| Diseases . | Phenotype . | Mechanism(s) . | Effective Tx . | Relative frequency, % . |
|---|---|---|---|---|
| Abbreviations: MGUS, monoclonal gammopathy of undetermined significance; SLE, systemic lupus erythematosus; Tx, treatment | ||||
| Lymphoproliferative diseases | 38 | |||
| MGUS IgG | Type 2 | Autoantibody | IVIg | |
| MGUS IgM | Type 2 | Unknown | None | |
| Myeloproliferative diseases | ||||
| Primary thrombosis | Type 2 | Increased proteolysis | Platelet reduction | 17 |
| Neoplasia | 5 | |||
| Wilm tumor | Type 1–3 | Unknown | Tumor reduction | |
| Immune | 4 | |||
| SLE | Type 2 | Autoantibody | Prednisone, IVIg | |
| Cardiovascular | Mild type 2 | Incrased proteolysis | Surgical repair | 15 |
| Hypothyroidism | Type 1 | Decreased synthesis | Thyroid replacement | 5 |
| Miscellaneous | 16 | |||