Intravenous immunoglobulin in patients with acquired Von Willebrand syndrome: A single referral centre experience

Background Acquired von Willebrand syndrome (AVWS) has been associated with monoclonal gammopathy of undetermined significance (MGUS), with limited data on its management. Methods We conducted a systematic literature search in Medline (Ovid), Embase, and Scopus up to September 11, 2019, for studies reporting on the management of AVWS associated with MGUS (AVWS‐MGUS). Data on patient characteristics, laboratory parameters at presentation, and clinical and laboratory outcomes were extracted. Objectives To describe the clinical presentation and outcomes of different therapeutic approaches. Results Seventy‐five studies were included in the final review, for a total of 137 patients. Most patients had von Willebrand factor ristocetin cofactor activity <30 IU/dL (86.6%) and factor VIII levels <50 IU/dL (91.8%). Bleeding severity ranged from no bleeding (16.1%) to minor bleeding (46.4%) and major bleeding (37.5%). The overall clinical success rates for 1‐deamino‐8‐D‐arginine vasopressin (DDAVP), factor replacement therapy, and intravenous immunoglobulin (IVIG) were 43.8%, 33.3%, and 85.4%, respectively. The laboratory response rates for DDAVP, factor replacement therapy, and IVIG were 39.0%, 62.9%, and 88.6%, respectively. Several other treatments were also reported in small numbers, out of which myeloma‐directed therapies, plasma exchange, recombinant factor VIIa, and antifibrinolytics appeared most successful, while immunosuppressive agents were largely ineffective. Conclusion IVIG appears to be an effective treatment for AVWS‐MGUS bleeding, conferring a high clinical success rate with measurable laboratory outcomes; albeit temporary. DDAVP and factor replacement therapy may be partially successful in controlling minor bleeds, but not major bleeds. Other less commonly used agents may be effective in certain cases, although data are limited.

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“… a All 11 patients were treated with IVIG in Bertolino et al 30 ; unable to determine reason for treatment from text …”

Section: Resultsmentioning

confidence: 99%

Acquired von Willebrand syndrome in monoclonal gammopathy – A scoping review on hemostatic management

Abou‐Ismail

Rodgers

Bray

et al. 2021

Research and Practice in Thrombosis and Haemostasis

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“…3 In contrast, FVIII: C and VWF:RCO levels have been corrected in some patients for up to 21 days after infusion of a lower dose of IVIG of 0.4 g/kg for 1, 2, or 5 days. 3,6,12 Although no correlation between IVIG dose and treatment response was reported by Bertolino et al, 9 we suppose that reducing the dose of IVIG infusions may be of interest to avoid potential serious side effects relative to high dose of IVIG, such as anaphylaxis, acute renal failure, or thromboembolic events. [14][15][16] Irrespective of the dose used, monitoring VWF:RCO, VWF:Ag, and FVIII:C levels after IVIG infusions is required to evaluate the extent and the duration of response to treatment.…”

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confidence: 88%

“…5,11 Recently, a retrospective study reported that no response to IVIG was noticed in only three of 17 of AVWS patients: high IgG monoclonal paraproteins were observed for two patients (18 and 45 g/L) and serum protein electrophoresis revealed an IgA associated to an IgM in the third patient. 12 The patient reported herein, who was diagnosed with AVWS in the context of IgG-MGUS, was successfully treated with IVIG at a dose of 1 g/kg/day during 2 successive days according to the ISTH recommendations. 5,7 When treatment with IVIG is effective in restoring normal VWF levels, the duration of response to treatment is variable.…”

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confidence: 99%

“…5,7 When treatment with IVIG is effective in restoring normal VWF levels, the duration of response to treatment is variable. 3,[5][6][7][8][9]12 Several authors have reported an increase of FVIII:C and VWF:RCO levels the day after the second infusion of high-dose IVIG, reaching maximal value on day 4, and progressively decreasing to baseline levels within 3 weeks. Persistent normalization of FVIII:C and VWF:RCO levels was only obtained in patients who received repetitive doses of IVIG.…”

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confidence: 99%

“…Conversely, a retrospective study highlighted that a biological response to IVIG was always observed in patients with levels of IgG monoclonal paraprotein under 10 g/L, whereas no biological response was observed in two others patients with higher IgG monoclonal paraprotein measurements (18 and 45 g/L). 12 Whether the dose of IVIG influences the duration of response to treatment is also debatable. Federici reported that lower doses of IVIG (single infusion of 0.5 g/kg) are less effective to improve clotting factor levels.…”

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confidence: 99%

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A Prolonged Treatment Response in Acquired Von Willebrand Syndrome

et al. 2019

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Acquired von Willebrand syndrome (AVWS) is a rare bleeding disorder. We report herein a case of AVWS due to a monoclonal gammopathy of undetermined significance, in which a transient but prolonged response to a treatment by intravenous immunoglobulin (IVIG) was observed. The diagnosis was fortuitously made in a preoperative setting for neurosurgery, after biological exploration of an isolated prolonged activated partial thromboplastin time. AVWS was confirmed by an accelerated clearance of an infused plasma-derived von Willebrand factor (VWF) concentrate. High doses of IVIG were used to perform the neurosurgery. Fifty-four days after IVIG, the patient was still responding to treatment with normal levels of factor VIII and VWF.

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Coagulation Complications of Cancer Patients

Wang¹,

Sanfilippo²

2022

Holland‐Frei Cancer Medicine

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Overview Patients with cancer have increased risks of coagulation complications including bleeding and/or thrombotic problems, which can be a result of malignancy itself and/or related to cancer therapies. These complications can lead to increased morbidity and mortality, as well as delays or alterations in cancer treatment. Therefore, identification of risk factors, preventation, and treatment strategies are important priorities in the care of cancer patients. In this chapter, we discussed and summarized relevant literature in common bleeding and thrombotic complications, their presentations, risk factors, prevention and management strategies.

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Intravenous immunoglobulin in patients with acquired Von Willebrand syndrome: A single referral centre experience

Cited by 3 publications

References 5 publications

Acquired von Willebrand syndrome in monoclonal gammopathy – A scoping review on hemostatic management

Acquired von Willebrand syndrome in monoclonal gammopathy – A scoping review on hemostatic management

A Prolonged Treatment Response in Acquired Von Willebrand Syndrome

Coagulation Complications of Cancer Patients

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