Pathogenesis (disease development)
Willebrand-Jürgens syndrome refers to a defect or deficiency of von Willebrand factor (vWF). For the various forms of Willebrand-Jürgens syndrome, see “Introduction.”
Etiology (Causes)
Biographic causes
- Genetic burden from parents, grandparents.
Disease-related causes (= acquired).
Blood, blood-forming organs – immune system (D50-D90).
- Autoimmunological diseases, unspecified
Neoplasms – tumor diseases (C00-D48).
- Malignant lymphoma (malignant neoplasm of the lymphatic system), including B-cell lymphomas.
- Monoclonal gammopathy – paraproteinemia with the appearance of monoclonal antibodies.
- Myeloproliferative neoplasms (MPN) (formerly chronic myeloproliferative disorders (CMPE)):
- Acute Di Guglielmo syndrome (erythremia).
- Chronic myeloid leukemia (CML)
- Essential thrombocythemia (ET) – chronic myeloproliferative disorder (CMPE, CMPN) characterized by chronic elevation of platelets (thrombocytes).
- Osteomyelofibrosis/osteomyelosclerosis (OMF or OMS).
- Polycythaemia vera (rubra) (PV)
- Non-Hodgkin’s lymphoma (NHL), including hairy cell leukemia (name comes from the frayed B cells detectable in bone marrow).
- Plasmocytoma (multiple myeloma).
Drugs
- Valproic acid – active substance used in epilepsy.