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  • 1
    Electronic Resource
    Electronic Resource
    Guidelines for the diagnosis and management of von Willebrand disease in Italy (2002)
    Oxford, UK : Blackwell Science Ltd
    Haemophilia 8 (2002), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary.  von Willebrand disease (vWD) is a bleeding disorder caused by quantitative (type 1 and 3) or qualitative (type 2) defects of von Willebrand factor (vWF). The molecular basis of type 2And 3 vWD are now known and those of type 1 vWD are being understood. Phenotypic diagnosis is based on the measurements of plasma and platelet vWF, of the ability of vWF to interact with platelet receptors and the analysis of the multimeric structure of vWF. Due to the heterogeneity of vWF defects and the variables that interfere with vWF levels, a correct diagnosis of types and subtypes may sometimes be difficult but is very important for therapy. The aim of treatment is to correct the dual defects of haemostasis, i.e. abnormal intrinsic coagulation expressed by low levels of factor VIII (FVIII) and abnormal platelet adhesion. Desmopressin is the treatment of choice in patients with type 1 vWD, who account for approximately 70% of cases, because it corrects FVIII–vWF levels and the prolonged bleeding time (BT) in the majority of these patients. In type 3 and in severe forms of type 1 and 2 vWD patients, desmopressin is not effective and it is necessary to resort to plasma concentrates containing FVIII and vWF. Treated with virucidal methods, these concentrates are effective and safe, but they cannot always correct BT defect. Platelet concentrates or desmopressin can be used as adjunctive treatments when poor correction of BT after plasma concentrate treatment is associated with continued bleeding.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2516.2002.00672.x
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  • 2
    Electronic Resource
    Electronic Resource
    Desmopressin and type II B von Willebrand disease (1996)
    Oxford, UK : Blackwell Publishing Ltd
    Haemophilia 2 (1996), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary. Type II B von Willebrand disease (vWD) is a rare subtype of vWD characterized by the presence of an abnormal von Willebrand factor (vWF) with enhanced affinity for the platelet membrane receptor glycoprotein Ib. The phenotypic hallmarks of the disease are represented by heightened ristocetin-induced platelet aggregation, occurring at very low ristocetin concentration, and the lack of high-molecular-weight vWF multimers in plasma. When infused with desmopressin, a variable degree of thrombocytopenia usually occurs in these patients, resulting from in vivo platelet aggregation caused by the release of abnormal vWF multimers from endogenous stores. We have reviewed the available literature data concerning the biological and clinical effects of desmopressin in the few cases so far reported. Despite the fear of thrombotic or haemorrhagic events, no significant side-effects have been reported also in the cases (70%) with severe thrombocytopenia. Moreover, the few clinical reports with the use of desmopressin in type II B patients showed a favourable effect in the prevention of bleeding during surgery or dental extraction. Thus, it appears that desmopressin could be safely used in selected clinical situations in patients with type II B vWD.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2516.1996.tb00018.x
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  • 3
    Electronic Resource
    Electronic Resource
    Acquired haemophilia: experience of two Italian centres with 17 new cases (1997)
    Oxford, UK : Blackwell Science Ltd
    Haemophilia 3 (1997), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary. Acquired haemophilia is a rare but often catastrophic haemorrhagic disorder associated with a high mortality rate. No single therapeutic approach has been consistently successful and clinical experience remains mainly anecdotal. This report describes 17 new cases diagnosed at two Italian haemophilia centres between 1979 and 1995.There was no difference in sex distribution. Mean age at diagnosis was 50 years. Fifty-nine per cent of cases had associated disorders and 29% developed an inhibitor post-partum. Eleven (64%) patients required substitutive therapy. Desmopressin was successfully used in five cases for minor bleeding. Immunosuppressive drugs (steroid, cyclophosphamide or experimental therapy) were used in 14 (82%) cases. Eight of 15 (52%) evaluable cases achieved complete remission (four post-partum). Fatal haemorrhage occurred in 2/15 (13%) of patients within 2 days from diagnosis.Acquired haemophilia is a severe coagulopathy. Prompt diagnosis with characterization and intensive treatment is usually required. However, particular subsets of patients such as those with inhibitor occurring post-partum or with low inhibitor titre at diagnosis usually show a more favourable clinical outcome.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2516.1997.00102.x
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  • 4
    Electronic Resource
    Electronic Resource
    Successful pregnancy in a woman with congenital factor XIII deficiency treated with substitutive therapy (1987)
    Springer
    Annals of hematology 55 (1987), S. 45-48 
    Details
    ISSN: 1432-0584
    Keywords: Factor XIII deficiency ; Recurrent abortion ; Pregnancy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A syndrome of marked fetal wastage is associated with congenital factor XIII deficiency in adult women. A previously unreported case of a woman with factor XIII deficiency is described, in which substitutive treatment with normal plasma or placental factor XIII concentrate permitted two normal pregnancies. Factor XIII activity was maintained above 1–2% with intermittent infusion of 300 ml to 450 ml of plasma every 14 days or of 500 units of concentrate every 21 days. This case confirms the only other case so far reported in which factor XIII substitutive therapy was able to permit a normal pregnancy in a woman with factor XIII deficiency and seems to suggest factor XIII to be involved in the process of annidation.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00319641
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  • 5
    Electronic Resource
    Electronic Resource
    Erythema multiforme after intravenous immunoglobulin (1988)
    Springer
    Annals of hematology 56 (1988), S. 145-145 
    Details
    ISSN: 1432-0584
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00320024
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  • 6
    Electronic Resource
    Electronic Resource
    Clinical effectiveness of desmopressin in a case of acquired von Willebrand's syndrome associated with benign monoclonal gammopathy (1989)
    Springer
    Annals of hematology 58 (1989), S. 211-213 
    Details
    ISSN: 1432-0584
    Keywords: Acquired von Willebrand's syndrome ; DDAVP ; Desmopressin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A case of acquired von Willebrand's syndrome (avWs) secondary to benign monoclonal gammopathy, is described, in which desmopressin (DDAVP) has proven effective repeatedly in preventing bleeding after tooth extraction. The laboratory pattern was similar to that of congenital type IA von Willebrand's disease. After DDAVP, prolonged bleeding time and factor VIII/von Willebrand factor activities were normalized. The disappearance rate of the elicited activities was similar to that observed in patients with congenital disease. This report adds to the scarce data concerning the haemostatic effectiveness of DDAVP in avWs and suggests that this agent might also be used in controlling or preventing bleeding in patients with the acquired disease, selected on the basis of their biological responsiveness to a test-infusion.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00320776
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  • 7
    Electronic Resource
    Electronic Resource
    Clinical significance of fibrinopeptide A in acute lymphocytic and non-lymphocytic leukaemia (1989)
    Springer
    Annals of hematology 59 (1989), S. 177-183 
    Details
    ISSN: 1432-0584
    Keywords: Acute leukaemia ; Disseminated intravascular coagulation ; Fibrinopeptide A ; Heparin treatment
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Fibrinopeptide A (FPA) was systematically investigated in 74 patients with acute leukaemia at different stages of the disease (50 with non-lymphocytic leukaemia, ANLL; 24 with lymphocytic leukaemia, ALL). At diagnosis, 75% of the cases had high FPA levels (86% in ANLL and 54% in ALL) with significantly higher levels in ANLL than in ALL (13.4 vs 4.4 ng/ml; p〈0.001). Patients with DIC (20 cases in ANLL and 1 case in ALL) had significantly higher levels (p〈0.001). FPA levels were neither correlated with fibrinogen or FDP levels nor with blast cell count. During chemotherapy, median FPA did not show significant changes whereas, at the end of therapy, a return toward normality was generally observed both in ALL and ANLL apart from the group of patients with acute promyelocytic leukaemia. Among the 24 patients who entered post-remission follow-up (13 ANLL and 11 ALL), 10 cases out of the 11 relapsing (6/6 with ANLL and 4/5 with ALL) had increased FPA 1 to 2 months before the ascertainment of the relapse. However, 16% and 9% of the samples obtained on different occasions, respectively from ANLL and ALL cases in maintained first remission, showed FPA above the normal limit. This study demonstrates that subclinical activation of blood coagulation, as indicated by high FPA level, is common both in lymphocytic and non-lymphocytic leukemia and suggests that this phenomenon is related to disease activity.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00320064
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