1.FVIII inhibitor surveillance in children with hemophilia A in Indonesia:a report from the Indonesian Pediatric Hematology-Oncology Working Group
Novie Amelia CHOZIE ; Djajadiman GATOT ; Bambang SUDARMANTO ; Susi SUSANAH ; Rini PURNAMASARI ; Pudjo Hagung WIDJAJANTO ; Susanto NUGROHO ; Olga RASIYANTI ; Dian PUSPITASARI ; Muhammad RIZA ; Maria Christina SHANTY LARASATI ; Sri Suryo ADIYANTI ; Made Citra SARASWATI ; Fitri PRIMACAKTI ; On behalf of the Pediatric Hematology-Oncology Working Group of the Indonesian Pediatric Society
Blood Research 2022;57(4):272-277
Background:
Factor VIII (FVIII) inhibitor diagnosis and surveillance in Indonesia are challenging owing to geographic conditions and the lack of laboratory facilities nationwide for inhibitor assays. This study aimed to determine the prevalence of FVIII inhibitors in children diagnosed with hemophilia A (HA) in Indonesia.
Methods:
A cross-sectional study was conducted in 12 hospitals in eight provinces of Indonesia between 2020 and 2021. Factor VIII inhibitor screening was performed in a central hemostasis laboratory for all children with HA (≤18 yr) who had received a minimum of 10 exposure days to clotting factor concentrates. The FVIII inhibitor titer was determined using the Bethesda assay.
Results:
Children (388) were enrolled in this study, including 219 (56.4%), 131 (33.8%), and 38 (9.4%) with severe, moderate, and mild HA, respectively. The prevalence of children who developed FVIII inhibitors was 37 out of 388 (9.6%). Factor VIII inhibitors were found in 25/219 (11.4%) severe, 11/131 (8.3%) moderate, and 1/38 (2.6%) children with mild HA. Thirteen children had low-titer inhibitors and 24 had high-titer inhibitors, with a median of 9.44 (1.48‒412.0) Bethesda Units. Among 13 children with low-titer inhibitors, eight underwent a confirmation test, of which five tested negative and were classified as transient. A significant difference in annual joint bleeding rate was found between patients with low and high inhibitor titers and those without inhibitors (P <0.001).
Conclusion
Factor VIII inhibitor prevalence in Indonesia was relatively low. However, the risk factors that may contribute to FVIII inhibitor development among Indonesian patients require further study.
2.Caspase 3 Expressions in Children with Acute Lymphoblastic Leukemia During Induction Phase Chemotherapy
Lukman Oktadianto ; Mia Ratwita Andarsini ; I Dewa Gede Ugrasena ; Yetti Hernaningsih ; Andi Cahyadi ; Maria Christina Shanty Larasati
Malaysian Journal of Medicine and Health Sciences 2023;19(No.3):91-97
Introduction: Caspase-3 is a crucial mediator of the extrinsic apoptosis pathway. The role of caspase-3 for extrinsic
apoptosis signalling is still a challenge and should be exploited in childhood ALL. This study aimed to compare
the caspase-3 expression in the patient’s bone marrow before and after the induction phase of chemotherapy in
childhood ALL. It will also to correlate the mean difference in caspase-3 expression between ALL standard-risk and
ALL high-risk patients. Methods: Seventeen newly diagnosed ALL subjects were enrolled in this study. Caspase-3
expression in bone marrow was assessed using flow cytometry and monoclonal antibodies. A T-test and a paired
T-test were used to compare between groups. The correlation coefficient between ALL groups was evaluated using
Spearman’s test and linear regression with a significant p-value of 0.05. Results: The caspase-3 expression is higher
after induction therapy. However, it showed an insignificant difference (16.56+12.91% vs 27.71+12.33%; p = 0.08,
p > 0.05). The mean difference of caspase-3 in ALL high-risk groups was significantly higher than in ALL standard-risk
groups with a positive correlation (p = 0.007, r = 0.756). Conclusion: The caspase-3 expression after induction phase
chemotherapy was increased in all standard-risk and high-risk patients; other lymphoblast apoptosis markers need to
be confirmed alongside caspase-3.