Willebrand-Jürgens Syndrome: Causes

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