Modern management of severe platelet function disorders

Alamelu, Jayanthi; Liesner, Ri

doi:10.1111/j.1365-2141.2010.08191.x

Cited by 85 publications

(119 citation statements)

References 94 publications

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“…It has been reported that anti-fibrinolytics [19], the recombinant factor VIIa [20], and vasopressin [21], which stimulates the release of factor VII, may be useful for treatment of severe bleedings in patients with BSS. Although platelet transfusion was omitted, these regents may also support the hemostasis [22]. In addition, to the best of our knowledge, this is the first case to be treated with cytotoxic chemotherapy for malignant tumor with BSS.…”

Section: Discussionmentioning

confidence: 88%

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

Okita¹,

Hihara²,

Konishi³

et al. 2010

Breast Cancer

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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.

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Section: Discussionmentioning

confidence: 88%

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

Okita¹,

Hihara²,

Konishi³

et al. 2010

Breast Cancer

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“…Bone marrow transplantation is the treatment to cure the disorder, although it has only been performed in a few cases with clinically severe phenotype [41].…”

Section: Stem Cell Transplantationmentioning

confidence: 99%

Glanzmann’s thrombasthenia – newer treatment options

Ghosh

Shetty

2013

Expert Opinion on Orphan Drugs

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“…In both of these disorders, significant mucocutaneous bleeding and ICH have been reported, although ICH is rare, occurring in only 0.3% to 2.0% of patients with GT and even less in those with BSS. 103,104 The PFA-100 is a fairly reliable screening mechanism for these diagnoses (Table 1). 102,103 Less well characterized but more common, the disorders of platelet signaling and secretion result from a variety of defects.…”

Section: Congenital Platelet Abnormalitiesmentioning

confidence: 99%

“…103,104 The PFA-100 is a fairly reliable screening mechanism for these diagnoses (Table 1). 102,103 Less well characterized but more common, the disorders of platelet signaling and secretion result from a variety of defects. Platelet activation leads to a conformational change in the platelet and normally results in secretion of platelet granule contents, which recruits other platelets to the site of injury.…”

Section: Congenital Platelet Abnormalitiesmentioning

confidence: 99%

Evaluating for Suspected Child Abuse: Conditions That Predispose to Bleeding

Carpenter¹,

Abshire²,

Anderst³

et al. 2013

Pediatrics

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Child abuse might be suspected when children present with cutaneous bruising, intracranial hemorrhage, or other manifestations of bleeding. In these cases, it is necessary to consider medical conditions that predispose to easy bleeding/bruising. When evaluating for the possibility of bleeding disorders and other conditions that predispose to hemorrhage, the pediatrician must consider the child’s presenting history, medical history, and physical examination findings before initiating a laboratory investigation. Many medical conditions can predispose to easy bleeding. Before ordering laboratory tests for a disease, it is useful to understand the biochemical basis and clinical presentation of the disorder, condition prevalence, and test characteristics. This technical report reviews the major medical conditions that predispose to bruising/bleeding and should be considered when evaluating for abusive injury.

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Modern management of severe platelet function disorders

Cited by 85 publications

References 94 publications

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

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

Glanzmann’s thrombasthenia – newer treatment options

Evaluating for Suspected Child Abuse: Conditions That Predispose to Bleeding

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