Bleeding complications after arthroscopy in a JAK2V617F-positive patient with essential thrombocythemia and acquired von Willebrand syndrome (AVWS)

Rupa‐Matysek, Joanna; Lewandowski, Krzysztof; Lewandowska, Maria; Wojtasińska, Ewelina; Wojtaszewska, Marzena; Walczak, Michał; Bykowska, Ksenia; Komarnicki, Mieczysław

doi:10.1007/s12185-014-1707-7

Cited by 7 publications

(5 citation statements)

References 27 publications

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“…It is not easy to recommend thromboprophylaxis in patients with AVWS caused by ET (especially with platelets >1 000 000 mmc −1 ), because these patients may have a bleeding tendency. On the other hand, their thrombotic risk is not negligible, as shown by reports of these complications after orthopaedic surgery in patients affected by ET associated with AVWS .…”

Section: Discussionmentioning

confidence: 97%

Orthopaedic surgery in patients with von Willebrand disease

et al. 2013

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Patients with von Willebrand disease (VWD) may need orthopaedic surgery because of disabling chronic arthropathy due to recurrent joint bleeding. They may also require this surgery independently of their haemostasis disorder. Knowledge regarding the management of orthopaedic surgery in VWD is limited. Description of management of orthopaedic surgery in patients with VWD, based upon retrospective data collection and analysis of 32 orthopaedic procedures carried out over a period of 33 years in 23 patients was the aim of this study. Of 32 procedures, six were minor (three hand surgery, one foot surgery, two others) and 26 were major (seven joint replacements, nine arthroscopic procedures, two foot surgery, eight others). Twenty-two procedures were performed using replacement therapy with plasma-derived concentrates containing both factor VIII (FVIII) and von Willebrand factor (VWF). Two procedures in patients with acquired von Willebrand syndrome (AWVS) were performed using FVIII-VWF concentrates associated with intravenous immunoglobulins, or desmopressin plus tranexamic acid. Seven procedures were performed using desmopressin alone and one using intravenous immunoglobulins in AVWS. Bleeding complications occurred in seven procedures (22%). In one patient, an anti-VWF antibody was diagnosed after surgery. Anticoagulant prophylaxis of venous thromboembolism was implemented in four cases only and in two instances there was excessive bleeding. In conclusion, control of surgical haemostasis was achieved in most patients with VWD undergoing orthopaedic surgery. The control of haemostasis combined with an adequate surgical technique and early post-operative rehabilitation are warranted for the successful performance of orthopaedic surgery in VWD, which requires the involvement of specialized haemophilia centres.

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Section: Discussionmentioning

confidence: 97%

Orthopaedic surgery in patients with von Willebrand disease

et al. 2013

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“…This correction of VWF:RCo after cytoreduction could be a reason why bleeding was uncommon, as most of the patients with ET and PV in this study were placed on cytoreductive treatment according to standard risk stratification. Most reports of major bleeding in patients with MPN with AVWS are based on anecdotes, mainly in patients with unrecognized AVWS [23][24][25][26]. These observations indicate that most hemorrhagic events induced by AVWS occur before or at the time of MPN diagnosis and that proper cytoreduction can minimize the risk of bleeding.…”

Section: Discussionmentioning

confidence: 99%

Acquired von willebrand syndrome in patients with philadelphia-negative myeloproliferative neoplasm

et al. 2023

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BackgroundAcquired von Willebrand syndrome (AVWS) has not been investigated in Korean patients with Philadelphia chromosome-negative myeloproliferative neoplasm. MethodsThis study analyzed the prevalence at diagnosis and clinical features of AVWS in patients with essential thrombocythemia (ET), polycythemia vera (PV), prefibrotic/early primary myelofibrosis (pre-PMF), or overt PMF (PMF) diagnosed between January 2019 and

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“…Joint bleeding has been rarely reported in AVWS [25]. Only a few cases of post-procedural [6] or post-traumatic [5][6][7] joint bleeding in AVWS have been reported in the literature, with only one report describing subsequent chronic synovitis [6], which was managed with arthroscopic synovectomy. In consideration of the anecdotal report of chronic synovitis in this subset of patients, evidence is scarce and a consensus on its management is lacking.…”

Section: Discussionmentioning

confidence: 99%

“…It presents with mucocutaneous bleeding [2] (bruising, epistaxis, menorrhagia, gastrointestinal bleeding and excessive post-surgical or postdental extraction blood loss). Compared to congenital hemophilia A and B, which are characterized by recurrent joint bleeding episodes [3] resulting in chronic arthropathy [4] and muscular hematomas, hemarthrosis in the course of AVWS is anecdotal and mainly and post-procedural or post-traumatic [5][6][7]. The established treatment of chronic synovitis in hemophilia is synovectomy, which can be surgical, chemical, or radioactive [8].…”

Section: Introductionmentioning

confidence: 99%

Successful Chemical Synovectomy in a Patient with Acquired von Willebrand Syndrome with Chronic Synovitis Due to Recurrent Knee Hemarthrosis: A Case Report

et al. 2022

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Acquired von Willebrand syndrome (AVWS) is a rare, non-hereditary bleeding disorder related to heterogeneous medical conditions such as hematological malignancies and cardiovascular and autoimmune diseases. We describe the clinical course of a 62-year-old man with polycythemia vera who experienced post-traumatic knee and leg swelling due to hemarthrosis. He was treated at another center with low molecular weight heparin due to misdiagnosed deep vein thrombosis further exacerbating the ongoing bleeding. At our center, he was diagnosed with AVWS with reduced von Willebrand factor (VWF):GPIbR plasma activity and loss of high molecular weight multimers (HMWM). He was treated with compressive bandages with resolution. Five months later, on clinical recurrence of knee and leg swelling, knee ultrasound scan showed the presence of chronic synovitis and a hemorrhagic Baker's cyst with signs of rupture. The treatment consisted of chemical synovectomy with rifampicin and steroids preceded by systemic replacement therapy using plasma-derived factor VIII-VWF concentrate. At the end of the treatment cycle, our patient reported complete resolution of knee pain and restoration of joint range of motion and function. Ultrasound evaluation confirmed complete resolution of knee capsule distension and Baker's cyst. Hemarthrosis is an anecdotal presentation of AVWS and chemical synovectomy was successful in treating this complication. A multidisciplinary approach allowed an effective management of this rare complication.

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Bleeding complications after arthroscopy in a JAK2V617F-positive patient with essential thrombocythemia and acquired von Willebrand syndrome (AVWS)

Cited by 7 publications

References 27 publications

Orthopaedic surgery in patients with von Willebrand disease

Orthopaedic surgery in patients with von Willebrand disease

Acquired von willebrand syndrome in patients with philadelphia-negative myeloproliferative neoplasm

Successful Chemical Synovectomy in a Patient with Acquired von Willebrand Syndrome with Chronic Synovitis Due to Recurrent Knee Hemarthrosis: A Case Report

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