지원사업
학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
커뮤니티
연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2021.12
- 수록면
- 315 - 321 (7page)
- DOI
- 10.5045/br.2021.2021165
이용수
초록· 키워드
오류제보하기
Background Glanzmann thrombasthenia is associated with abnormalities in the glycoprotein IIb/IIIa receptor. This study, conducted at Ampang Hospital, Malaysia, aimed to assess outcomes of blood management strategies for Glanzmann thrombasthenia.
Methods Ten patients with Glanzmann thrombasthenia aged 9 years (2009?2018) were examined.
Data on clinical characteristics, transfusion practices, and patient blood management were obtained from medical records. Patient blood management methods included parenteral iron, erythropoietin, hormonal pills, intrauterine progesterone contraceptive devices, tranexamic acid, and recombinant factor VIIa. Primary outcomes were hemoglobin levels and the proportion of patients who received blood transfusion. Secondary outcomes were morbidity and mortality.
Results The median age at diagnosis was 8.2 years (range, 1?15 yr). The female-to-male ratio was 9:1. Eight patients had type 2 disease (5?20% of normal GPIIb/IIIa), and two patients had type 1 disease (normal GPIIb/IIIa ＜5%). All patients had iron deficiency. All female patients presented with significant menorrhagia. Other bleeding symptoms included epistaxis, spontaneous skin bruising, hemoptysis, gingival bleeding, knee hemarthrosis, and pelvic hematoma. No patient experienced life-threatening bleeding. Our patients had a mean hemoglobin level of 5.6 g/dL at diagnosis. All patients were optimized using non-transfusion methods as described above. Our patient had a current mean hemoglobin level of 11 g/dL. Approximately 70% (7/10) of patients did not experience receiving blood transfusions in the last 5 years. No patient experienced non-transfusion-related morbidities such as sepsis, thromboembolism, or cardiorespiratory events.
Conclusion High cost, transfusion-related adverse events, and immunomodulation could be effectively prevented by avoiding unnecessary blood transfusions.
목차
등록된 정보가 없습니다.
참고문헌 (23)
참고문헌 신청
Pittman MA Jr / 1964 / Glanzmann’s thrombopathy : an autosomal recessive trait in one family / Am J Med Sci 247:293~303
Nurden AT / 2015 / Expanding the mutation spectrum affecting II3 integrin in Glanzmann thrombasthenia : screening of the ITGA2B and ITGB3 genes in a large international cohort / Hum Mutat 36:548~561
Tholouli E / 2004 / Acquired Glanzmann’s thrombasthenia without thrombocytopenia : a severe acquired autoimmune bleeding disorder / Br J Haematol 127:209~213
Nurden AT / 2011 / Glanzmann thrombasthenia : a review of ITGA2B and ITGB3 defects with emphasis on variants, phenotypic variability, and mouse models / Blood 118:5996~6005
Miller JL / 2009 / Glycoprotein analysis for the diagnostic evaluation of platelet disorders / Semin Thromb Hemost 35:224~232
Nurden AT / 2015 / Expanding the mutation spectrum affecting II3 integrin in Glanzmann thrombasthenia : screening of the ITGA2B and ITGB3 genes in a large international cohort / Hum Mutat 36:548~561
Tholouli E / 2004 / Acquired Glanzmann’s thrombasthenia without thrombocytopenia : a severe acquired autoimmune bleeding disorder / Br J Haematol 127:209~213
Nurden AT / 2011 / Glanzmann thrombasthenia : a review of ITGA2B and ITGB3 defects with emphasis on variants, phenotypic variability, and mouse models / Blood 118:5996~6005
Miller JL / 2009 / Glycoprotein analysis for the diagnostic evaluation of platelet disorders / Semin Thromb Hemost 35:224~232
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