Detection of alloimmunization in Glanzmann Thrombasthenia and Bernard-Soulier Syndrome: Data from a Brazilian Center

Gabe, Caroline; Ziza, Karen Chinoca; Durazzo, Natália; Pagani, Flavia M.; Oliveira, Vítor Hugo de; Conrado, Marina-C.A.V.; Dezan, Marcia Regina; Mendrone, Alfredo; Villaça, Paula Ribeiro; Dinardo, Carla Luana; Rocha, Vanderson

doi:10.1016/j.htct.2022.06.005

Cited by 3 publications

(14 citation statements)

References 20 publications

(28 reference statements)

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“…It involves a biochemical defect that proposes low or no amount of the glycoprotein Ib-IX-V complex 6 . This complex has two important functions, such as mediating adhesion to the blood vessel wall and facilitating the platelet activation capacity of thrombin at lower levels, which explains the longer bleeding time observed in patients with BSS, a condition that corroborates the present report 1,7 .…”

Section: Discussionsupporting

confidence: 91%

“…It is more often misdiagnosed as immune thrombocytopenia, which leads to unnecessary interventions such as splenectomy 6,7 . Its prevalence, according to the literature, can be estimated at less than 1 in 1 million 1 . It involves a biochemical defect that proposes low or no amount of the glycoprotein Ib-IX-V complex 6 .…”

Section: Discussionmentioning

confidence: 99%

“…The orientation about the indispensability of avoiding any kind of trauma or antiplatelet medication is indispensable. 1,6 If platelet deficiency or other bleeding dyscrasias are suspected, immediate referral for hematological evaluation is indicated, as in this case 4 . The management of this group of patients should consider the severity of the disease, the invasiveness of the planned dental procedure, and the experience of the professional responsible for the treatment 1,5 .…”

Section: Discussionmentioning

confidence: 99%

“…Bernard-Soulier syndrome (BSS), also known as thrombocytic hemorrhagic dystrophy, is a disease that is associated with autosomal recessive inheritance, characterized by the presence of extremely large platelets and prolonged bleeding time 1 . This syndrome was first described in 1948 by Bernard and Soulier, where they report the case of a male patient who died at the age of 28 due to intracranial hemorrhage after a fight².…”

Section: Introductionmentioning

confidence: 99%

“…Thus, while in congenital disorders associated with platelet dysfunction, the most frequently employed therapies in this group of patients are platelet transfusions in more severe cases, and the use of desmopressin and/or antifibrinolytic drugs in mild cases. Sometimes, the application of alternative factors and therapies is necessary, both in the preparation prior to the treatment of the patient, and in rescue therapies after intense or prolonged bleeding, for example, the reserve of fresh frozen plasma if bleeding in the transoperative period 1,3,5 . This paper aims to present an alternative management of surgical dental procedures in a female patient with Bernard-Soulier Syndrome.…”

Section: Introductionmentioning

confidence: 99%

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Manejo cirúrgico em paciente com síndrome de Bernard-Soulier: relato de caso

Pereira¹,

Silva²,

Santos³

et al. 2023

ISJHR

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Bernard-Soulier syndrome is a rare inherited disease characterized by thrombocytopenia, presence of extremely large platelets and prolonged bleeding time. Objective: This paper aims to present an alternative management of surgical dental procedures in a female patient, faioderma, 27 years old, carrier of Bernard-Soulier syndrome. Case Description: On clinical examination and history, the patient reported a history of recurrent pericoronaritis associated with units 38 and 48, and a history of bleeding associated with menstruation. Imaging examination revealed impacted and impacted dental units. The treatment plan included exodontia of elements 18, 28, 38, and 48 under general anesthesia, transfusion of platelet concentrates (01U apheresis or 07U pool) two hours before surgery, fresh frozen plasma reserve in case of transoperative bleeding, and RBC concentrate reserve, according to the guidelines prescribed by the hematologist. At the moment, in a seven-month postoperative follow-up, the patient evolves without signs and symptoms of coagulation disorders associated with exodontia. Conclusion: Thus, it is essential that dental professionals are aware of the multidisciplinary management strategies for this group of patients in order to minimize postoperative complications.

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Section: Discussionsupporting

confidence: 91%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Manejo cirúrgico em paciente com síndrome de Bernard-Soulier: relato de caso

Pereira¹,

Silva²,

Santos³

et al. 2023

ISJHR

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Should HLA and HPA‐matched platelet transfusions for patients with Glanzmann Thrombasthenia or Bernard‐Soulier syndrome be standardized care? A Dutch survey and recommendations

Huisman,

Holle,

Schipperus

et al. 2024

Transfusion

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BackgroundGlanzmann thrombasthenia (GT) and Bernard‐Soulier syndrome (BSS) patients require frequent platelet transfusions and hence have an increased risk for alloimmunization against donor Human Leukocyte Antigens (HLA) when no HLA‐matching is performed. Knowing that Human Platelet Antigens (HPA) are located on the platelet glycoproteins that can be absent in these patients, preventive HPA‐matching may also be considered. Uniform recommendations on this topic lack in transfusion guidelines making standard practice unclear, therefore, we aimed to provide a framework for matched platelet transfusions.Study Design and MethodsWe conducted a targeted literature search and a national survey of Dutch (pediatric) hematologists from July to September 2021.ResultsWe found 20 articles describing platelet transfusion policies in 483 GT‐patients and 29 BSS‐patients, both adults and children. Twenty surveys were returned for full analysis. All responders treated patients with platelet disorders, including GT (n = 36 reported) and BSS (n = 29 reported). Of respondents, 75% estimated the risk of antibody formation as “likely” for HLA and 65% for HPA. Formation of HLA antibodies was reported in 5 GT and in 5 BSS‐patients, including one child. Fifteen respondents gave preventive HLA‐matched platelets in elective setting (75%). Three respondents additionally matched for HPA in GT‐patients (15%). Main argument for matched platelet transfusions was preventing alloimmunization to safeguard the effectivity of ‘random’ donor‐platelets in acute settings.ConclusionElective HLA‐matching for GT and BSS‐patients is already conducted by most Dutch (pediatric) hematologists. HPA‐matching is mainly applied when HPA‐antibodies are formed. Based on the current literature and the survey, recommendations are proposed.

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Imlifidase Utilization in Glanzmann Thrombasthenia with Anti-GPIIb/IIIa and Anti-HLA Alloimmunization and Severe Platelet Refractoriness following Hematopoietic Stem Cell Transplant.

AlNajjar,

Rochat,

Grimes

et al. 2024

Preprint

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Glanzmann Thrombasthenia (GT) is an inherited bleeding disorder of poor platelet function secondary to a defect in platelet membrane glycoprotein IIb/IIIa (GPIIb/IIIa). Patients with GT may develop anti-platelet antibodies including anti-GPIIb/IIIa which can lead to severe refractory thrombocytopenia and life-threatening bleeding, management of which is challenging. We report successful use of imlifidase, a novel IgG protease enzyme, as part of a multimodal approach for management of severe platelet refractoriness and alloimmunization in a child with GT and primary graft failure following hematopoietic stem cell transplant (HSCT). The patient had no detectable anti-platelet alloantibodies following imlifidase, and underwent a second HSCT.

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Detection of alloimmunization in Glanzmann Thrombasthenia and Bernard-Soulier Syndrome: Data from a Brazilian Center

Cited by 3 publications

References 20 publications

Manejo cirúrgico em paciente com síndrome de Bernard-Soulier: relato de caso

Manejo cirúrgico em paciente com síndrome de Bernard-Soulier: relato de caso

Should HLA and HPA‐matched platelet transfusions for patients with Glanzmann Thrombasthenia or Bernard‐Soulier syndrome be standardized care? A Dutch survey and recommendations

Imlifidase Utilization in Glanzmann Thrombasthenia with Anti-GPIIb/IIIa and Anti-HLA Alloimmunization and Severe Platelet Refractoriness following Hematopoietic Stem Cell Transplant.

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