title: Cyclical thrombocytosis, acquired von Willebrand syndrome and aggressive non-melanoma skin cancers are common in patients with Philadelphia-negative myeloproliferative neoplasms treated with hydroxyurea.
abstract: Abstract Cyclical thrombocytosis, acquired von Willebrand syndrome, aggressive non-melanoma skin cancers and other hydroxyurea complications have been reported in Philadelphia-negative myeloproliferative neoplasms (MPNs), but their incidence and clinical consequences have not been defined in a large cohort of patients. We conducted a retrospective analysis of 188 consecutive patients with MPNs specifically addressing the incidence of these complications. Cyclical thrombocytosis was documented in 29 patients (15%), the majority of whom were receiving hydroxyurea. Acquired von Willebrand syndrome was identified in 17 of the 84 screened patients (20%), but was not associated with any major bleeding complications. Non-melanoma skin cancers were reported in 51 patients (27%). Hydroxyurea-related fever occurred in nine of 149 patients (6%) who received hydroxyurea. Seventy-three patients (39%) experienced a total of 98 major thrombotic events, with the majority of these occurring prior to or within 3 months of the diagnosis. Cyclical thrombocytosis, acquired von Willebrand syndrome, aggressive non-melanoma skin cancers and other hydroxyurea-related complications are not infrequent in MPNs and have important clinical consequences for management.
authors.affiliation: Department of Haematology, Concord Repatriation General Hospital , Concord, NSW , Australia.
authors.forename: Emma
authors.initials: E
authors.lastname: Verner
authors.forename: Cecily
authors.initials: C
authors.lastname: Forsyth
authors.forename: Andrew
authors.initials: A
authors.lastname: Grigg
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D006918:Hydroxyurea / Q000009:adverse effects* / Q000627:therapeutic use; D053614:Janus Kinase 2 / Q000235:genetics; D054438:Leukemia, Myeloid, Chronic, Atypical, BCR-ABL Negative / Q000150:complications* / Q000188:drug therapy / Q000235:genetics; D008297:Male; D008875:Middle Aged; D009196:Myeloproliferative Disorders; D010507:Periodicity; D010976:Platelet Count; D012878:Skin Neoplasms / Q000175:diagnosis / Q000209:etiology*; D013922:Thrombocytosis / Q000175:diagnosis / Q000209:etiology*; D013927:Thrombosis / Q000209:etiology; D014842:von Willebrand Diseases / Q000175:diagnosis / Q000209:etiology*
chemical_list: D053614:Janus Kinase 2; D006918:Hydroxyurea