Maintaining hemostasis in acquired von <scp>W</scp>illebrand syndrome: a review of intravenous immunoglobulin and the importance of rituximab dose scheduling

Kanakry, Jennifer A.; Gladstone, Douglas E.

doi:10.1111/trf.12017

Cited by 13 publications

(13 citation statements)

References 46 publications

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“…31 Prednisone, other immunosuppressants and rituximab have been described in the literature with varying results, it is unclear whether they work by modulating the activity of the antibodies against VWF or by controlling the potential underlying disorder. 32 Association with cardiovascular disorders. A variety of cardiovascular disorders have been associated with AVWS and can include congenital defects, acquired valve and other structural abnormalities and intracardiac devices.…”

Section: Acquired Von Willebrand Syndrome (Avws)mentioning

confidence: 99%

Acquired bleeding disorders in the elderly

Kruse‐Jarres

2015

Hematology

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The hemostatic balance changes with advancing age which may be due to factors such as platelet activation, increase of certain clotting factor proteins, slowing of the fibrinolytic system, and modification of the endothelium and blood flow. Generally, this predisposes the elderly to thrombosis rather than bleeding. It often necessitates antiplatelet or anticoagulation therapy, which can cause significant bleeding problems in an aging population. Additionally, changing renal function, modification in immune regulation, and a multitude of other disease processes, can give rise to acquired bleeding disorders. Bleeding can prove difficult to treat in a dynamic environment and in a population that may have underlying thrombotic risk factors.This article discusses some specific challenges of acquired bleeding arising in the elderly. The use of anticoagulation and nonsteroidal anti-inflammatory medications is prevalent in the treatment of the elderly and predisposes them to increased bleeding risk as their physiology changes. When prescribing and monitoring these therapies, it is exceedingly important to weigh thrombotic versus bleeding risks. There are additional rare acquired bleeding disorders that predominantly affect the elderly. One of them is acquired hemophilia, which is an autoimmune disorder arising from antibodies against factor VIII. The treatment challenge rests in the use of hemostatic agents in a population that is already at increased risk for thrombotic complications. Another rare disorder of intensifying interest, acquired von Willebrand syndrome, has a multitude of etiologic mechanisms. Understanding the underlying pathophysiology is essential in making a treatment decision for this disorder. Learning Objectives• To recognize that coagulation changes with age and that it may predispose elderly patients on antithrombotic therapies to bleeding • To recognize the underlying thrombotic risk and comorbidities complicating the treatment of acquired hemophilia A • To understand the various pathophysiologic mechanisms leading to Acquired Von Willebrand Syndrome and assign appropriate treatment according to that mechanism Acquired bleeding disorders in the aging population are of concern because they are not readily diagnosed. In a study, community physicians from various specialties were presented with a hypothetical older adult woman complaining of recurrent epistaxis. Although most practitioners ordered complete blood counts and activated partial thromboplastin time (aPTT) as part of their work-up, less than one-half of nonhematologists repeated a significantly prolonged aPTT and Ͻ45% consulted a hematologist. Of those, emergency medicine physicians were least likely to consult hematology. 1

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Section: Acquired Von Willebrand Syndrome (Avws)mentioning

confidence: 99%

Acquired bleeding disorders in the elderly

Kruse‐Jarres

2015

Hematology

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“…In the present case, although SMZL was quite advanced, the patient refused to receive conventional chemotherapy and instead chose rituximab monotherapy. In the literature, rituximab has not been effective in AVWS associated with MGUS (15-17) or with lymphoplasmacytic lymphoma (18), although it has been effective in AVWS associated with B cell lymphoma (MALT lymphoma) (10,11), monoclonal B cell lymphocytosis (19), essential mixed cryoglobulinemia (20), and SLE (21,22). Although the lymphadenopathy in our case showed mild shrinkage, the AVWS was not resolved.…”

Section: Discussionmentioning

confidence: 99%

Splenic Marginal Zone Lymphoma with Acquired von Willebrand Syndrome Diagnosed via Splenic Bleeding

et al. 2017

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An 85-year-old woman underwent emergent splenectomy due to left abdominal pain and active bleeding in a massively enlarged spleen. The histological diagnosis was splenic marginal zone lymphoma (SMZL). A prolonged activated partial thromboplastin time (APTT) was noted, and additional tests led to the diagnosis of type 2A-like acquired von Willebrand syndrome (AVWS). An APTT cross mixing test ruled out the presence of inhibitors. She received eight courses of rituximab monotherapy. The coagulation data showed no improvement, possibly because the lymphoma showed a poor response to the treatment. AVWS rarely causes bleeding in solid organs. This is the first case of SMZL with AVWS diagnosed via splenic bleeding.

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“…A diagnosis of acquired VWS was established and the patient was treated with high-dose IVIg obtaining the stop of bleeding within 48 hours. One month after IVIg, he was treated with rituximab for four weekly doses followed by eight doses of maintenance, and no further bleeding occurred [22]. …”

Section: Discussionmentioning

confidence: 99%

Salvage therapy with high dose Intravenous Immunoglobulins in acquired Von Willebrand Syndrome and unresponsive severe intestinal bleeding

et al. 2014

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A 91-year-old woman affected with acquired Von Willebrand (VW) syndrome and intestinal angiodysplasias presented with severe gastrointestinal bleeding (hemoglobin 5 g/dl). Despite replacement therapy with VW factor/factor VIII concentrate qid, bleeding did not stop (eleven packed red blood cell units were transfused over three days). High circulating levels of anti-VW factor immunoglobulin M were documented immunoenzimatically. Heart ultrasound showed abnormalities of the mitral and aortic valves with severe flow alterations. When intravenous immunoglobulins were added to therapy, prompt clinical and laboratory responses occurred: complete cessation of bleeding, raise in hemoglobin, VW factor antigen, VW ristocetin cofactor and factor VIII levels as well as progressive reduction of the anti-VWF autoantibody levels.

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Maintaining hemostasis in acquired von Willebrand syndrome: a review of intravenous immunoglobulin and the importance of rituximab dose scheduling

Abstract: A maintenance regimen of rituximab may be an effective long-term management strategy for AVWS associated with lymphoproliferative disorders, which may minimize the use of blood products and IVIG.

Cited by 13 publications

References 46 publications

Acquired bleeding disorders in the elderly

Acquired bleeding disorders in the elderly

Splenic Marginal Zone Lymphoma with Acquired von Willebrand Syndrome Diagnosed via Splenic Bleeding

Salvage therapy with high dose Intravenous Immunoglobulins in acquired Von Willebrand Syndrome and unresponsive severe intestinal bleeding

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