Blood Res 2021; 56(4):
Published online December 31, 2021
https://doi.org/10.5045/br.2021.2021165
© The Korean Society of Hematology
Correspondence to : Ganesh Kasinathan, MB BCh BAO, MRCPI
Department of Hematology, Ampang Hospital, Jalan Mewah Utara, Pandan Mewah, Ampang, Selangor 68000, Malaysia
E-mail: ganeshkasinathan11@hotmail.com
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
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.
Keywords Glanzmann thrombasthenia, Hemoglobin, Erythropoietin, Menorrhagia, Iron
Blood Res 2021; 56(4): 315-321
Published online December 31, 2021 https://doi.org/10.5045/br.2021.2021165
Copyright © The Korean Society of Hematology.
Ganesh Kasinathan, Jameela Sathar
Department of Hematology, Ampang Hospital, Selangor, Malaysia
Correspondence to:Ganesh Kasinathan, MB BCh BAO, MRCPI
Department of Hematology, Ampang Hospital, Jalan Mewah Utara, Pandan Mewah, Ampang, Selangor 68000, Malaysia
E-mail: ganeshkasinathan11@hotmail.com
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
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.
Keywords: Glanzmann thrombasthenia, Hemoglobin, Erythropoietin, Menorrhagia, Iron
Table 1 . Patient’s demographic characteristics and baseline clinical variables..
Demographic characteristics | Values |
---|---|
Gender | |
Female, N (%) | 9 (90) |
Male, N (%) | 1 (10) |
Age (yr) | |
Mean (SD) | 35.2 (6.8) |
Min-Max | 27–49 |
Ethnicity | |
Malay, N (%) | 7 (70) |
Chinese, N (%) | 1 (10) |
Indian, N (%) | 2 (20) |
Others, N (%) | 0 (0) |
Family history of bleeding | |
Yes, N (%) | 8 (80) |
No, N (%) | 2 (20) |
Baseline Hb (g/dL) | |
Mean (SD) | 5.7 (1.1) |
Baseline platelet (×109/L) | |
Mean (SD) | 222 (59.6) |
Age of diagnosis (yr) | |
Mean (SD) | 8.2 (4.7) |
Parental consanguinity | |
Yes, N | 6 |
No, N | 4 |
Iron deficiency | |
Yes, N | 10 |
No, N | 0 |
Abbreviations: max, maximum; min, minimum; n, number; SD, standard deviation..
Table 2 . Incidence of bleeding symptoms..
Clinical features | N of patients (%) |
---|---|
Menorrhagia | 9/9 (100) |
Gingival bleeding | 8/10 (80) |
Epistaxis | 3/10 (30) |
Skin bruises (spontaneous) | 2/10 (20) |
Pelvic hematoma | 1/10 (10) |
Hemoptysis | 2/10 (20) |
Hemarthrosis (knee) | 1/10 (10) |
Upper gastrointestinal bleeding | 0/10 (0) |
Intracranial bleeding | 0/10 (0) |
Table 3 . Non-transfusion PBM therapy..
Non-transfusion PBM therapies | N (%) |
---|---|
Tranexamic acid | 10 (100) |
Intravenous iron | 10 (100) |
Erythropoiesis-stimulating agents | 1 (10) |
Hormonal therapy | |
Combined oral contraceptives | 8 (80) |
Etonogestrel implant | 1 (10) |
Progestin-intrauterine system | 1 (10) |
Recombinant factor VIIa | 6 (60) |
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