Successful perioperative management for a breast cancer patient with Bernard–Soulier syndrome

Abstract

We herein report the first case of a Bernard-Soulier syndrome (BSS) patient undergoing a surgical procedure for breast cancer. BSS is a rare hereditary thrombocytopathy associated with defects of the platelet glycoprotein complex glycoprotein Ib/V/IX and characterized by large platelets, thrombocytopenia, and severe bleeding symptoms. Because of the rarity of BSS, there are as yet no defined protocols for the perioperative management, which can be very complex and challenging in patients with coagulopathies, in particular BSS. In this case we successfully performed both an interventional examination as well as mastectomy with axillary lymph node dissection, under the preventive and intermittent transfusion of platelets. No intra- or postoperative bleeding complications occurred. Unfortunately, the patient was diagnosed as having metastatic disease involving liver, lungs, and bones 10 months after the surgery. She had received 1st, 2nd, and 3rd line chemotherapy without severe adverse events. However, gastrointestinal bleeding appeared after she was treated with 4th line chemotherapy. Finally, she succumbed 22 months after the breast surgery.

Utilization of paravertebral nerve blocks as part of a multimodal analgesic regimen in a patient with Bernard-Soulier syndrome undergoing a Nuss procedure

“…Case reports involving patients with BSS include off-pump cardiac bypass [6], breast surgery [7], endodontic surgery [8], total hip arthroplasty [9], laparotomy [10], and mandibular osteotomy [11]. In most cases, preoperative thrombocytopenia is present and corrected via platelet transfusion prior to the surgery.…”

“…In most cases, preoperative thrombocytopenia is present and corrected via platelet transfusion prior to the surgery. Further intraoperative transfusions are used based on the clinical impression of the patient's hematologic status and extent of blood loss related to the surgery [6,7,9,10]. There is a lack of evidence guiding a perioperative plan in patients presenting with a history of mild symptoms associated with BSS and normal platelet count, but prophylactic transfusion of platelets has been utilized [8,11].…”

Obstetric and gynecological intervention in women with Bernard-Soulier syndrome: Report of two cases

“…Има ју ћи у ви ду ма ли број бо ле сни ка са БСС, не по сто је пре ци зни про то ко ли при пре ме за раз ли чи те хи рур шке за хва те [1,69]. Иако се при ме на rF VI Ia по ка за ла ве о ма ефи ка сном и си гур ном код осо ба са Глан цма но вим (Glan zmann) син дро мом, ис ку ства код бо ле сни ка са БСС су ве о ма огра ни че на [1,10,11,12]. Сто га је пре по ру ка да бо ле сни ке са БСС тре ба при пре ми ти тран сфу зи јом тром бо ци та не по сред но пре опе ра ци је (4-6 до за кон цен тро ва них тром бо ци та, од но сно 1-2 пу ла, ако је мо гу ће HLAпо ду дар них тром бо ци та) и на ста ви ти по сто пе ра ци о но по по тре би [1,69].…”