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Titolo:
Acquired von Willebrand syndromes: clinical features, aetiology, pathophysiology, classification and management
Autore:
Michiels, JJ; Budde, U; van der Planken, M; van Vliet, HHDM; Schroyens, W; Berneman, Z;
Indirizzi:
Univ Antwerp Hosp, Dept Haematol, Antwerp, Belgium Univ Antwerp Hosp Antwerp Belgium Hosp, Dept Haematol, Antwerp, Belgium Int Haemostasis Thrombosis Sci Ctr, Goodheart Inst, Rotterdam, NetherlandsInt Haemostasis Thrombosis Sci Ctr Rotterdam Netherlands m, Netherlands Coagulat Lab, Hamburg, Germany Coagulat Lab Hamburg GermanyCoagulat Lab, Hamburg, Germany Univ Antwerp Hosp, Haematol Lab, Dept Biol Clin, Antwerp, Belgium Univ Antwerp Hosp Antwerp Belgium Lab, Dept Biol Clin, Antwerp, Belgium Univ Rotterdam Hosp, Haemostasis & Thrombosis Lab, Dept Haematol, Rotterdam, Netherlands Univ Rotterdam Hosp Rotterdam Netherlands matol, Rotterdam, Netherlands
Titolo Testata:
BEST PRACTICE & RESEARCH CLINICAL HAEMATOLOGY
fascicolo: 2, volume: 14, anno: 2001,
pagine: 401 - 436
SICI:
1521-6926(200106)14:2<401:AVWSCF>2.0.ZU;2-P
Fonte:
ISI
Lingua:
ENG
Soggetto:
INTRAVENOUS GAMMA-GLOBULIN; BLEEDING GASTROINTESTINAL ANGIODYSPLASIA; REGRESSION FOLLOWING TREATMENT; BENIGN MONOCLONAL GAMMOPATHY; SYSTEMIC-LUPUS-ERYTHEMATOSUS; RISTOCETIN COFACTOR ACTIVITY; II VONWILLEBRANDS DISEASE; ESSENTIAL THROMBOCYTHEMIA; FACTOR-VIII; HYDROXYETHYL STARCH;
Keywords:
acquired von Willebrand syndrome; von Willebrand factor; monoclonal gammopathy; multiple myeloma; macroglobulinaemia; lymphoproliferative disorders; non-Hodgkin's lymphoma; chronic lymphocytic leukaemia; thrombocythaemia; myeloproliferative disorders;
Tipo documento:
Review
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
148
Recensione:
Indirizzi per estratti:
Indirizzo: Michiels, JJ Univ Antwerp Hosp, Dept Haematol, Antwerp, Belgium Univ Antwerp Hosp Antwerp Belgium ematol, Antwerp, Belgium
Citazione:
J.J. Michiels et al., "Acquired von Willebrand syndromes: clinical features, aetiology, pathophysiology, classification and management", BEST P R C, 14(2), 2001, pp. 401-436

Abstract

Acquired von Willebrand syndrome (AVWS) associated with hypothyroidism is of type I, results from a decreased synthesis of factor VIII and von Willebrand factor (VWF), responds to desmopressin with normal half-life times forfactor VIII and VWF parameters, and disappears after treatment with I-thyroxine. AVWS type I or III, which occurs in a minority of patients with Wilms' tumour in the complete absence of an inhibitor against VWF and no absorption of factor VIII or VWF onto nephroblastoma cells, responds to chemotherapy and/or tumour resection. Hyaluronic acid produced by nephroblastoma cells may be the causative factor in atypical AVWS in Wilms' tumour. AVWS associated with thrombocythaemia of various myeloproliferative disorders is characterized by normal factor VIII and von Willebrand factor antigen (VWF:Ag)levels and a selective deficiency of functional ristocetin co-factor activity (VWF: RCo) and collagen-binding activity (VWF:CBA). AVWS type II in thrombocythaemia is caused by a platelet-dependent proteolysis of large VWF multimers, given the inverserelationship between platelet count and large VWFmultimers in plasma and specific increases in the number of proteolytic VWF fragments in plasma. The laboratory findings of AVWS associated with systemic lupus erythematosus or IgG benign monoclonal gammopathy are characterized by a prolonged bleeding time and activated partial thromboplastin time, decreased or absent ristocetin-induced platelet activity, low to very low levels of Factor VIII coagulant activity (mean 15%), VWF:Ag (mean 10.7%) and VWF:RCo (mean 6.2%),and a type II multimeric pattern of VWF. Neutralizing and nonneutralizing anti-VWF autoantibodies, usually IgG, have been detected in patient plasma either free or tightly bound to the intermediate and high molecular weight VWF factor VIII particles. The bound auto antibody-antigen complex is rapidly cleared from the circulation, resulting in low levels of factor VIII, VWF parameters as documented by a poor response to desmopressin and VWF factor VIII concentrate. High-dose intravenous immunoglobulin transiently corrects the Factor VIII coagulant and VWF levels, lasting For a few weeks in AVWS type II associated with systemic lupus erythematosus or IgG benign monoclonal gammopathy. Prednisolone is effective in AVWS associated with autoimmune disorder. Prednisolone and chemotherapy will not affect AVWS associated with IgG benign monoclonal gammopathy because the monoclonal IgG protein remains to act as an anti-VWF autoantibody. An absorption of VWF to malignant cells has been documented in a few patients with various lymphoproliferative disorders or adrenal carcinoma and suggested to result in a depletion of VWF. The clinical picture of AVWS associated with early-stage IgG multiple myeloma, chronic lymphocytic leukaemia or non-Hodgkin's lymphoma without a paraprotein or no detectable underlying disorder is similar to that of AVWS type II in IgG benign monoclonal gammopathy but poorly documented with regard to the underlying immune mechanism of AVWS. The mechanical destruction of large VWF multimers may be of relevance in conditions in which the shear rate of flowing blood is increased, as may occur in cases of aortic stenosis, other heart valve defects or stenosed vessels. Drug-induced AVWS has been described in association with the useof pesticides valproic acid, ciprofloxacin, griseofulvin, tetracycline, thrombolytic agents and hydroxyethyl starch.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 07/08/20 alle ore 07:08:38