Case report: 1-deamino-8-D-arginine vasopressin and cryoprecipitate in variant von Willebrand disease

1985 ◽  
Vol 20 (2) ◽  
pp. 169-173 ◽  
Author(s):  
Wahid T. Hanna ◽  
Joseph Slywka ◽  
Judith Dent ◽  
Zaverio M. Ruggeri ◽  
Theodore S. Zimmerman
Keyword(s):  
Case Report ◽  
Arginine Vasopressin ◽  
Von Willebrand Disease ◽  
Von Willebrand

scholarly journals Case report of von willebrand disease after tonsillotomy and adenotomy in a child

2021 ◽  
Vol 20 (1) ◽  
pp. 89-92
Author(s):  
S. S. Makhmudov ◽  
◽  
A. A. Ochilzoda ◽  
F. P. Dzhamolov ◽  
A. Z. Mutalibov ◽  
...  
Keyword(s):  
Case Report ◽  
Clinical Practice ◽  
Surgical Treatment ◽  
Clinical Case ◽  
Clinical Manifestations ◽  
Von Willebrand Disease ◽  
Rare Occurrence ◽  
Von Willebrand

The authors present a clinical case of von Willebrand disease detected after tonsillotomy and adenotomy in a child. The peculiarity of this observation lies in the rare occurrence of von Willebrand disease in clinical practice, as well as the manifestation of the clinical manifestations of this pathology against the background of surgical treatment in a child.

Spontaneous pyohaemothorax in a teenager with von Willebrand disease: a case report and review of literature

2021 ◽  
Vol 14 (8) ◽  
pp. e241613
Author(s):  
Vaishnavi Divya Nagarajan ◽  
Asha Shenoi ◽  
Lucy Burgess ◽  
Vlad C Radulescu
Keyword(s):  
Case Report ◽  
Von Willebrand Factor ◽  
Von Willebrand Disease ◽  
Surgical Drainage ◽  
Review Of Literature ◽  
History Of ◽  
Factor Concentrate ◽  
Type 3 ◽  
Von Willebrand ◽  
Willebrand Factor

An 18-year-old man with a history of type 3 von Willebrand disease (VWD) presented with a spontaneous pyohaemothorax. Type 3 VWD may present with both mucocutaneous and deep-seated bleeds, such as visceral haemorrhages, intracranial bleeds and haemarthrosis. There have been very few cases described in children of spontaneous pyohaemothorax. Management of this patient was challenging due to risks of bleeding following surgical drainage, requiring constant replacement with von Willebrand factor concentrate, while monitoring factor VIII levels to balance the risks of thrombosis.

scholarly journals von Willebrand disease with G4022A mutation (vWd Sungnam): a case report

1999 ◽  
Vol 14 (1) ◽  
pp. 93
Author(s):  
Kyung Soon Song ◽  
Shin Heh Kang ◽  
Myung Seo Kang ◽  
Young Sook Park ◽  
Jong Rak Choi ◽  
...  
Keyword(s):  
Case Report ◽  
Von Willebrand Disease ◽  
Von Willebrand

scholarly journals A Case Report of Aortic Valve Replacement for a Patient with von Willebrand Disease

2015 ◽  
Vol 44 (5) ◽  
pp. 292-295 ◽  
Author(s):  
Kazue Nakashima ◽  
Yukihiro Yoshimura ◽  
Shuji Toyama ◽  
Yoshiyuki Maekawa ◽  
Tadanori Minagawa ◽  
...  
Keyword(s):  
Case Report ◽  
Aortic Valve ◽  
Aortic Valve Replacement ◽  
Valve Replacement ◽  
Von Willebrand Disease ◽  
Von Willebrand

scholarly journals Desmopressin: a nontransfusional form of treatment for congenital and acquired bleeding disorders [see comments]

Blood ◽  
1988 ◽  
Vol 72 (5) ◽  
pp. 1449-1455 ◽  
Author(s):  
PM Mannucci
Keyword(s):  
Arginine Vasopressin ◽  
Von Willebrand Disease ◽  
Synthetic Analogue ◽  
Bleeding Disorders ◽  
Spinal Fusion Surgery ◽  
Prolonged Bleeding Time ◽  
Normal Individuals ◽  
Transfusion Requirements ◽  
Coagulant Activity ◽  
Von Willebrand

Abstract Desmopressin (1-deamino-8-D-arginine vasopressin, abbreviated DDAVP) is a synthetic analogue of the antidiuretic hormone L-arginine vasopressin. Because it can raise circulating levels of factor VIII coagulant activity (FVIII) and von Willebrand factor and shorten the prolonged bleeding time, DDAVP is established as a nontransfusional form of treatment for mild and moderate hemophilia and von Willebrand disease. Recently, DDAVP has also been purported to be useful for shortening the prolonged skin bleeding times that occur with uremia, cirrhosis, and platelet dysfunctions of various etiologies. Finally, there is evidence from controlled clinical trials that DDAVP can reduce blood loss and transfusion requirements for hemostatically normal individuals undergoing spinal fusion surgery and for patients undergoing cardiopulmonary bypass surgery. The purpose of this report is to review the therapeutic applications of DDAVP in congenital and acquired bleeding disorders and to discuss areas in which further basic and clinical research is needed.

Use of Recombinant Factor VIIa (rVIIa) for Treatment of Bleeding in Type II B Von Willebrand Disease: A Case Report

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 4514-4514
Author(s):  
Arash Rezazadeh ◽  
Patricia P Ashby ◽  
Vivek R. Sharma
Keyword(s):  
Case Report ◽  
Hormonal Therapy ◽  
Von Willebrand Disease ◽  
Coagulation Cascade ◽  
Factor Vii ◽  
Severe Reaction ◽  
Platelet Surface ◽  
First Case ◽  
Gi Bleed ◽  
Von Willebrand

Abstract Background: Von Willebrand disease (vWD) is an inherited bleeding disorder caused by a deficiency or dysfunction of von Willebrand’s factor (VWF). Type 2 vWD is a qualitative deficiency of VWF with the IIB variant being caused by a “gain of function” mutation that results in increased affinity of the vWF for platelet GPIb. Treatment of choice for bleeding episodes is factor VIII-VWF concentrate or DDAVP in selected cases. We are reporting a case of type IIB vWD where rVIIa was used successfully for hemostasis. Case report: A 28 year old white female with type IIB vWD had being doing well with Humate-P® which she used most frequently for menorrhagia. She had also been treated successfully with Humate-P® for peri-operative hemostasis during back surgery but approximately 3 years after this developed anaphylactic reaction while getting humate-P for an episode of severe menorrhagia. A test dose of a different lot of Humate-P® in the hospital also resulted in a severe reaction. Administration of Koâte®-DVI and Alphanate® was subsequently attempted but caused similar anaphylactic reactions after the first few doses. She had normal IgA levels. Amicar and hormonal therapy was used to control the menorrhagia to where she was able to maintain a relatively stable Hgb level and not require frequent PRBC transfusion. Unfortunately, a few months later the patient developed GI bleeding from what was initially an occult source and ended up needing upwards of 40 units of PRBC transfusion over a period of two months. She was offered options of trying cryoprecipitate as well as DDAVP but refused after weighing the risks and benefits. At this time, rVIIa was given and the bleeding appeared to slow down resulting in lesser frequency of transfusion but it did not entirely stop. After several unsuccessful attempts to locate the source, a tagged RBC study finally found the bleed originating near the cecal area which eventually turned out to be a malignant ileocecal carcinoid that had been missed on initial colonoscopy as well as on capsule camera endoscopy. There was no clincally overt carcinoid syndrome. Patient underwent a right hemicolectomy and received rVIIa perioperatively according to the protocol approved for patients with hemophilia with factor inhibitor. She received NovoSeven® 90 mcg/kg before the surgery and then q2h for the first 24 hours. She had rVIIa q3h on post-op day one. The dose frequency was dropped to q4h on day 2 followed by q6h on post-op days 3 to 7. This provided excellent perioperative hemostasis. She has done well with no further GI bleed or recurrence of the tumor after more than a year and her menorrhagia is fairly well controlled with hormonal therapy and amicar. Discussion: Factor VII, a vitamin K-dependent glycoprotein, promotes hemostasis by activating the extrinsic pathway of the coagulation cascade. Although it does not affect the vWF level it probably bypasses the deficiency via thrombin generation on platelet surface. In literature search we found two case reports regarding utilizing rFVII in vWD, both with uncontrolled bleeding despite adequate factor replacement. First case with acquired vWD and GI bleed and the second case was inherited vWD with GI angiodysplasia. The precise mechanism of action in this setting however is not fully understood. Conclusion: To our knowledge this is the first report of utilizing rVIIa in a patient intolerant to vWF without inhibitors to vWF. rVIIa should be considered a viable option for hemostasis in patients unable to tolerate vWF due to severe allergy.

scholarly journals Acute retroperitoneal hemorrhage after oocyte retrieval: a case report

2021 ◽  
Vol 2021 (3) ◽  
Author(s):  
Yangying Xu ◽  
Cuifang Hao ◽  
Xiaoqiang Liu ◽  
Zongzhi Yang ◽  
Xianhua Sun
Keyword(s):  
Case Report ◽  
Diagnostic Laparoscopy ◽  
Oocyte Retrieval ◽  
Von Willebrand Disease ◽  
Retroperitoneal Hemorrhage ◽  
Vitro Fertilization ◽  
History Of ◽  
Von Willebrand ◽  
Ivf Et

Abstract A 29-year-old woman with a 5-year history of primary infertility underwent in vitro fertilization-embryo transfer (IVF-ET) treatment. Hemorrhagic shock caused by retroperitoneal hematoma after oocyte retrieval was treated promptly by the evaluation of diagnostic laparoscopy and angiography. The patient was recovered and discharged from the hospital 7 days later without any complications. She was later diagnosed with Von Willebrand disease by a hematologist.

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