Perioperative intravenous immunoglobulin treatment in a patient with severe acquired von Willebrand syndrome: case report and review of the literature

Jennes, Eva; Guggenberger, Dorothee; Zotz, Rainer B.; Thompson, Lora M. A.; Brümmendorf, Tim H.; Koschmieder, Steffen; Jost, Edgar

doi:10.1002/ccr3.890

Cited by 6 publications

(5 citation statements)

References 37 publications

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“…Suspicion of AVWS was raised due to prolonged bleeding after surgical procedure or trephine biopsy of the bone marrow [8,13,14]. Sometimes an increasing tendency to bleed is the first sign of haematological disorder, which leads to the discovery of multiple myeloma, as in the presented case [11,12].…”

Section: Discussionmentioning

confidence: 79%

“…Some studies have shown that reduction of bleeding symptoms is not always connected with change in monoclonal IgG or VWF:Ag measurements. Moreover, treatment of the haematological disorder does not always result in improvement of AVWS manifestation [11,34].…”

Section: Discussionmentioning

confidence: 99%

“…According to data from the International Registry, 16 out of 186 (9%) AVWS patients were diagnosed with multiple myeloma. Additionally, in the literature there are approximately 30 reported cases of these two disorders in combination [8,9,10,11,12,13,14].…”

Section: Discussionmentioning

confidence: 99%

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Acquired von Willebrand syndrome associated with plasma cell myeloma – a rare histopathological bone marrow image

Chraszczewska

Sokół

Kuczkiewicz-Siemion

et al. 2019

pjp

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Acquired von Willebrand syndrome (AVWS) is a rare bleeding disorder that is associated with a variety of underlying diseases. We report a case of AVWS associated with plasma cell myeloma. The patient was a 57-year-old male with recurrent bleeding symptoms for a few months. Physical examination was normal. Laboratory studies revealed isolated prolongation of the activated partial thromboplastin time. His factor VIII activity, von Willebrand factor (VWF) Ag, and VWF activity were low. Bone marrow aspirate showed diffuse infiltration of atypical plasma cells and erythroid line hyperplasia.

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Section: Discussionmentioning

confidence: 79%

Section: Discussionmentioning

confidence: 99%

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Acquired von Willebrand syndrome associated with plasma cell myeloma – a rare histopathological bone marrow image

Chraszczewska

Sokół

Kuczkiewicz-Siemion

et al. 2019

pjp

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“…In our patient, rapid VWF clearance led to suspicion of an immune-mediated mechanism. Therefore, it was reasonable to use an immune-mediated treatment approach 1 12 13. This approach was successful even though VWF inhibitors or anti-VWF antibodies were not detected.…”

Section: Discussionmentioning

confidence: 99%

Surprise diagnosis of acquired von Willebrand syndrome in a patient previously thought to have type III von Willebrand disease: evaluation and periprocedural management

Zhou,

Kapoor,

Onwuemene

2023

BMJ Case Rep

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Acquired von Willebrand syndrome (AVWS) is a rare disorder that is characterised by an acquired deficiency of von Willebrand factor. AVWS was suspected in a patient with type III von Willebrand disease (VWD) who did not respond to factor replacement therapy. Given the crucial implications for management, we describe this patient’s clinical presentation, diagnosis and periprocedural management. To facilitate pericardiocentesis, periprocedural management included steroids, intravenous immunoglobulin and factor replacement therapy. In other patients with suspected immune-mediated AVWS, a similar approach may be effective. This case also highlights the importance of distinguishing AVWS from inherited VWD.

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“…Occasionally, immune-mediated AWVS can be treated with intravenous immunoglobulin with good bleeding control. [64][65][66] Factor XI Deficiency Factor XI deficiency is an uncommon autosomal bleeding disorder with variable severity of bleeding phenotypes, affecting both sexes. Since factor XI is involved in propagation and maintenance of coagulation rather than in the initiation of the coagulation cascade, patients with this disorder do not bleed spontaneously.…”

Section: Acquired Von Willebrand Syndromementioning

confidence: 99%

Hemostasis and Thrombosis in the Oldest Old

Tzoran

Hoffman

Monréal

2018

Semin Thromb Hemost

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There is a growing proportion of the elderly population in the Western world, and these individuals require special considerations regarding a broad variety of aspects, including treatment approaches to illnesses that affect all age groups. The hemostatic system in individuals changes considerably with aging. Specifically, changes in levels of procoagulant and natural anticoagulant factors along with thrombopathy simultaneously create a hypercoagulable state and hemostatic difficulties. Underlying morbidities, such as congestive heart failure, chronic obstructive pulmonary disease, diabetes mellitus, and cancer, increase the risk for venous and arterial thrombosis. This population is also increasingly affected by acquired bleeding disorders, including acquired hemophilia and acquired von Willebrand syndrome, as well as mild congenital bleeding disorders. Real-life data demonstrate that recurrent and fatal venous thromboembolism is the major hemostatic concern in the elderly. The fact that treatment of thrombotic complications increases the bleeding risk also has to be taken into consideration, particularly in the older age group. This remains true in the era of direct oral anticoagulants. In conclusion, maintaining a delicate balance between thrombosis and bleeding risks is the key issue in providing qualified treatment to elderly patients.

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Perioperative intravenous immunoglobulin treatment in a patient with severe acquired von Willebrand syndrome: case report and review of the literature

Cited by 6 publications

References 37 publications

Acquired von Willebrand syndrome associated with plasma cell myeloma – a rare histopathological bone marrow image

Acquired von Willebrand syndrome associated with plasma cell myeloma – a rare histopathological bone marrow image

Surprise diagnosis of acquired von Willebrand syndrome in a patient previously thought to have type III von Willebrand disease: evaluation and periprocedural management

Hemostasis and Thrombosis in the Oldest Old

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