title: Studies of von Willebrand factor in essential thrombocythemia patients treated with alpha-2b recombinant interferon.
abstract: The crucial role of the von Willebrand Factor (vWF) and its interaction with platelets in myeloproliferative disorders (MPD) have emerged in recent years. Recently, many authors have reported the therapeutical efficacy of interferon (IFN) in MPD with thrombocytosis in decreasing platelet number. The purpose of our report is to study the modifications of vWF in a series of 20 patients affected by essential thrombocythemia (ET) or MPD with thrombocytosis, treated with alpha 2b recombinant IFN (alpha 2b-rIFN). Patients were studied before treatment and after complete or partial response: vWF-related properties, bleeding time (BT) and ristocetin-induced platelet aggregation (RIPA) were evaluated. Before treatment, we found prolonged BT in 5 patients (25%), abnormal RIPA in 8 (40%), reduced factor VIII coagulant activity (VIII:C) in 2 (10%), reduced vWF-related antigen (vWF:Ag) in 5 (25%) and low vWF:ristocetin cofactor (vWF:Ricof) in 5 (25%). Twelve subjects were evaluated after hematologic remission: in all patients, BT, VIII:C, vWF:Ag and vWF:Ricof were within normal range or upper normal limits. RIPA was abnormal in 7 subjects. Multimer patterns of vWF were performed in 3 patients before and after treatment: 2 of them showed loss of high-molecular-weight multimers that seemed to recover at remission. IFN seems to induce improvement of platelet number and their functions in MPD with thrombocytosis.
authors.affiliation: Department of Human Biopathology, University La Sapienza, Rome, Italy.
authors.forename: M G
authors.initials: MG
authors.lastname: Mazzucconi
authors.forename: A
authors.initials: A
authors.lastname: Ferrari
authors.forename: S
authors.initials: S
authors.lastname: Solinas
authors.forename: A
authors.initials: A
authors.lastname: Vitale
authors.forename: A
authors.initials: A
authors.lastname: Chistolini
authors.forename: A B
authors.initials: AB
authors.lastname: Federici
authors.forename: F
authors.initials: F
authors.lastname: Mandelli
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D000077190:Interferon alpha-2; D016898:Interferon-alpha / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D011994:Recombinant Proteins; D013920:Thrombocythemia, Essential / Q000097:blood / Q000188:drug therapy*; D014841:von Willebrand Factor / Q000378:metabolism*
chemical_list: D000077190:Interferon alpha-2; D016898:Interferon-alpha; D011994:Recombinant Proteins; D014841:von Willebrand Factor