Haemate^® P/Humate‐P^® for the treatment of von Willebrand disease: considerations for use and clinical experience

Abstract

von Willebrand disease (VWD) is a heterogeneous bleeding disorder with symptoms in affected patients ranging from mild effects to potentially devastating haemorrhagic events. Desmopressin (DDAVP) and von Willebrand factor/factor VIII (VWF/FVIII) concentrates are the principal treatments. Haemate P/Humate-P is an intermediate-purity VWF/FVIII concentrate with extensive clinical experience in VWD. This concentrate has been shown to correct haemostatic defects of VWD, with efficacy ratings of good/excellent in nearly all patients treated for bleeding or surgical events. Haemate P/Humate-P has a high content of the high molecular weight (HMW) VWF multimer fraction, which has been shown to be very effective in achieving haemostasis. The HMW VWF multimer pattern in Haemate P/Humate-P is more similar to that of normal human plasma (94% for Haemate P/Humate-P vs. 100% for normal human plasma) than that of other VWF/FVIII concentrates and correlates with functional VWF activities including ristocetin cofactor activity (VWF:RCo) and collagen-binding activity. The recommended dosing of Haemate P/Humate-P is based preferentially on VWF:RCo activity, which is approximately twice that of FVIII:C (2.4:1). Haemate P/Humate-P has been shown to be safe; no serious adverse events or cases of thrombosis have been observed in clinical trials and no documented cases of viral transmission in nearly three decades of clinical use. While DDAVP is effective in a large proportion of VWD patients, it may not provide adequate haemostasis in all situations. In such cases, Haemate P/Humate-P is an effective replacement concentrate for all types of VWD in both adult and paediatric patients.

Activated prothrombin complex concentrate factor VIII inhibitor bypassing activity (FEIBA) for the reversal of warfarin-induced coagulopathy

“…Therefore, many guidelines recommending PCC administration lack recommendations regarding the dosing regimen [9, 10, 18, 19]. In particular, the recommendations do not specify whether to use non-activated PCC preparations or, on the contrary, activated PCC preparations, known as anti-inhibitor coagulant complex or FEIBA (factor VIII inhibitor bypassing activity; available as FEIBA VH or Autoplex T) [22]. A recent trial concluded that an "individualized" dosage regimen of PCC based on target-INR, initial-INR and patient body weight is significantly more effective in reaching target INR than a fixed dose [23].…”

Caring for the Patient With von Willebrand Disease Who Requires Ambulatory Surgery

“…These agents have a long history of use with demonstrated efficacy with minimal side effects. 5 By having a greater awareness and comfort with vWD and how it is managed, the perianesthesia nurse can help to optimize surgical outcomes and avoid complications after ambulatory surgery.…”

“…10 Humate-P contains vWF as well as albumin and fibrinogen, which add to its efficacy in optimizing coagulation. 5 These agents are generally well tolerated, but if elevation of heart rate occurs, the infusion of the product should be slowed to optimize patient comfort. 5 Rarely a patient may show signs of an allergic hypersensitivity to the product, exhibiting symptoms such as pruritis, wheezing, hypotension, and chest tightness.…”

“…5 These agents are generally well tolerated, but if elevation of heart rate occurs, the infusion of the product should be slowed to optimize patient comfort. 5 Rarely a patient may show signs of an allergic hypersensitivity to the product, exhibiting symptoms such as pruritis, wheezing, hypotension, and chest tightness. 10 In these rare cases, the product should be stopped immediately and crystalloids and other treatments be provided as directed by the attending anesthesiologist or other physician.…”

Successful Ultrasound-Guided Femoral Nerve Blockade and Catheterization in a Patient with Von Willebrand Disease

“…Humate-P is a human plasma-derived vWF/FVIII concentrate with an extensive, three-decade track record of no thrombosis and no cases of viral transmission [12, 13]. It is recommended for surgical prophylaxis and spontaneous bleeding treatment in all types of vWD and in vWD cases that are refractory to desmopressin.…”