Thalassemias are heterogeneous group of genetic disorders. β-thalassemia is existed due to impaired production of beta globins chains, which leads to a relative excess of alpha globin chains. The abnormalities of haemoglobin synthesis are usually inherited but may also arise as a secondary manifestation of another disease, most commonly haematological neoplasia. This article presenting two cases of acquired β-thalassemia in children with ALL focusing on the diagnosis and the possible relationship between the two haematological diseases. The first case is a four (4) year old boy with ALL-L1 type at maintenance phase of chemotherapy, he suffered from anaemia with Hb 8.0 g/dL, WBC 22,600/mm3 and platelets count of 200,000/mm3, peripheral blood smear revealed anisocytosis, polychromes, hypochromia, basophilic stippling, and normoblastocytes. The result of Hb electrophoresis of Hb A of 54.9%, Hb F of 29.4%, Hb E of 13.4% and Hb A2 of 2.3%. The patient was diagnosed as ALL-L1 type and β-thalassemia. The second case, is a 13 year old girl with remission ALL-L1 type after chemotherapy, she suffered from anaemia with Hb 6.7 g/dL, WBC 12,400/mm3, platelet count was 200,000/mm3, and peripheral blood smear obtained anisocytosis, hypochromia, normoblastocytes, myelocytes and basophilic stippling. The result of Hb electrophoresis are: Hb F 0.41%, Hb A1c 0.78%, Hb A2 2.95% with the conclusion of a β-thalassemia trait, this patient was diagnosed with ALL-L1 type remission + β-thalassemia trait. The case reviewers assume that acquired β-thalassemia which happened in those patients were the altered expression of globin chain which mechanism for this syndrome might be the acquisition of a mutation that affects RNA or proteins involved in β-globin gene regulation and resulting the reduction of the (α/β)-globin biosynthetic ratios, or/and associated with chemotherapy-inducement.
Introduction: Anemia often occurs in critically ill children and is associated with increased morbidity and mortality in women and children and impaired cognitive and behavioral development in children. Objective: This study aims to understand the profile and characteristics of anemia patients in the critical care population. Materials and Methods: This is a retrospective, descriptive study of the patient's medical records. Data were collected by the total sampling technique. Results: Among 203 patients, 52% were anemic at admission to the Pediatric Intensive Care Unit (PICU), while 45% were anemic at discharge. Anemia tends to be more common in older age and male individuals with their chief complaints being respiratory symptoms, higher mean Red Cell Distribution Width (RDW) levels, and poorer nutritional status. There were 84 patients (41%) who received Packed Red-Cells (PRC) transfusions, among them there were 54 patients (51%) who were anemic at PICU admission. Of the 84 patients who received PRC transfusions during their PICU stay and 43 patients (47%) were anemic on PICU discharge. Conclusion: Anemia is quite common in critically ill children and is dominated by male patients aged under 5 years. Anemia also mostly happens in patients with higher organ dysfunction scores and poorer nutritional status than nonanemic patients. Half of the patients with anemia at the PICU also received PRC blood transfusion.
Background: Transfusion-dependent thalassemia (TDT) necessitates regular transfusions, resulting in complications such as iron overload, hemolytic anemia, and the emergence of alloantibodies/ autoantibodies. This situation poses challenges in obtaining compatible transfusions. Excessive iron and chronic hemolysis impact the elevation of Interleukin-6 (IL-6), initiating an inflammatory process that triggers hepcidin formation and influences antibody development. This study aims to analyze disparities in IL-6 and hepcidin levels and establish the correlation between IL-6 and hepcidin in TDT patients with and without alloimmunization/autoimmunization. Methods: Forty whole blood samples were collected from TDT patients with and without alloimmunization/autoimmunization, centrifuged, and the serum extracted, then stored in a refrigerator at -80°C. IL-6 and hepcidin levels were assessed using the ELISA method. The Mann-Whitney U test was employed to evaluate differences in hepcidin and IL-6 levels between the two groups. In contrast, the Spearman Correlation test was utilized to analyze the correlation between hepcidin and IL-6 levels. Results: IL-6 levels in the TDT group with alloimmunization/autoimmunization (3.64 pg/ mL) were significantly higher compared to the TDT group without alloimmunization/autoimmunization (1.41 pg/mL; p < 0.05). Hepcidin levels in the TDT group with alloimmunization/autoimmunization (2,950.6 pg/mL) were significantly higher compared to the TDT group without alloimmunization/autoimmunization (1,599.6 pg/mL; p < 0.05). The Spearman correlation test revealed a significant positive correlation between hepcidin and IL-6 levels in TDT patients with alloimmunization/autoimmunization (r = 0.764; p = 0.000). Additionally, a significant positive correlation was observed between hepcidin and IL-6 levels in TDT patients without alloimmunization/autoimmunization (r = 0.559; p = 0.010). Conclusion: IL-6 and hepcidin levels were elevated in TDT patients with alloimmunization/autoimmunization compared to those without. Interleukin-6 and hepcidin exhibited a positive correlation in both transfusion-dependent thalassemia groups.
Immune Thrombocytopenia Purpura (ITP) is an autoimmune disorder triggered by antiplatelet autoantibodies. Without prompt treatment and close monitoring, the condition could worsen, potentially resulting in fatal consequences. Clinically, ITP is classified into three phases including Newly-Diagnosed ITP, Persistent ITP, and Chronic ITP, each with distinct durations and therapy implications. In ITP patients, platelet counts decrease, accompanied by abnormal shifts in platelet indices, including Mean Platelet Volume (MPV), Platelet Distribution Width (PDW), Platelet Large Cell Ratio (P-LCR), and Plateletcrit (PCT). Thus, therapeutic response in ITP patients can be evaluated through increased platelet counts and normalization of platelet indices. This study aimed to assess the therapeutic response of platelet counts and platelet indices in pediatric ITP patients by comparing pre- and post- therapy levels. This retrospective study included ITP patients under 18 years old at Dr. Soetomo General Academic Hospital, Surabaya, conducted from September 2023 to March 2024. Platelet count and platelet indices pre- and post-therapy levels were analyzed using the Paired T-test for normally distributed data and the Wilcoxon test for non-normally distributed data, with significance set at p < 0.05. In summary, there were notable changes in the pre- and post-therapy levels of platelet, MPV, PDW, P-LCR, and PCT in each ITP category and for all therapies. Platelet count and PCT increased, while MPV, PDW, and P-LCR decreased. Patients treated with prednisone exhibited the best therapeutic response. Among the categories, Newly Diagnosed ITP demonstrated the most optimal therapeutic response. Overall, ITP therapy led to significant differences between pre- and post-therapy levels, marked by an increase in platelet counts and normalization of platelet indices.
Thalassemia, the fifth-most catastrophic disease with 10,555 patients, causes physical, emotional, and economic burden for the patient, their family, and the country. Annually, IDR 500 billion are needed to cover the treatment of thalassemia. This projected number will continue to increase if no action is taken, so education and knowledge dissemination are important for thalassemia prevention. This community development involves thalassemia patients and families as partners and encourages them to share their knowledge and experience about thalassemia with the Indonesian late adolescents, young adult, and middle-aged adult population through an online webinar to increase public knowledge on thalassemia. Education media, such as posters and videos, were developed and disseminated during the activity, followed by live sharing from thalassemia patients, parents, and pediatricians. The respondents’ knowledge of thalassemia was measured using an 11-items questionnaire before and after the online educational activity. The results show that the respondents have a good knowledge of thalassemia, and it increases after attending online education event about the disease (9.74+2.4, 10.22+0.77, p<0.001). Their knowledge was associated with health-related field experience, knowing someone with thalassemia, and their willingness to undergo thalassemia testing (p<0.046, 0.013, and 0.007, respectively). Thus, these findings support the importance of education and dissemination of information regarding Thalassemia. Moreover, strong knowledge might lead to a willingness to undergo thalassemia testing, which might lead to less marriage between carriers and, eventually, a reduction in the incidence of thalassemia major.
Over the past 10 years, infection has remained as the main cause of illness and mortality among children with Acute Lymphoblastic Leukemia on chemotherapy. The high incidence of Hospital-Acquired Pneumonia in children with Acute Lymphoblastic Leukemia on chemotherapy with risk factors should be intervened earlier.An observational case control study of children with Acute Lymphoblastic Leukemia on chemotherapy. Patient with Hospital-Acquired Pneumonia considered as case and patient without Hospital-Acquired Pneumonia as control to analyze risk factors that affect the incidence of Hospital-Acquired Pneumonia in children with Acute Lymphoblastic Leukemia on chemotherapy from 2016 to 2018 was performed in the pediatric ward Dr. Soetomo General Academic Hospital with a total sampling technique. Nine risk factors were analyzed: age, gender, nutritional status, length of stay, risk stratification, chemotherapy phase, anemia, neutropenia, and thrombocytopenia. Bivariate and multivariate analysis using chi-square, continuity correction, and logistic regression was used for statistical analysis.120 children enrolled the study. Analyzed of risk factors showed risk stratification (p = 0.009), chemotherapy phase (p < 0.001), and neutropenia (p < 0.001) was proven to significantly affect the incidence of Hospital-Acquired Pneumonia in children with Acute Lymphoblastic Leukemia on chemotherapy. Age, gender, nutritional status, length of stay, anemia, and thrombocytopenia were not proven to be a risk factor that affects the incidence of Hospital-Acquired Pneumonia in children with Acute Lymphoblastic Leukemia on chemotherapy.The incidence of Hospital-Acquired Pneumonia in children with Acute Lymphoblastic Leukemia on chemotherapy is significantly affected by the risk stratification, chemotherapy phase, and neutropenia.
Leukemia Limfoblastik Akut (LLA) adalah penyakit keganasan sel progenitor limfoid yang berasal dari sumsum tulang. Tanda khasdari diagnosis leukemia akut adalah sel blas. Pemeriksaan mikroskopis dilakukan untuk menentukan persentase sel blas pada diagnosisleukemia akut. Immunophenotyping merupakan metode diagnostik yang dapat membantu menegakkan diagnosis pada keganasanhematologi. CD34 merupakan antigen yang sering digunakan untuk identifikasi sel induk hemopoeisis atau blas. Penelitian ini bertujuanuntuk mengetahui kenasaban antara persentase blas dengan ekspresi CD34 di sumsum tulang di pasien leukemia limfoblastik akut anaksebelum dan sesudah pengobatan kemoterapi fase induksi.
Thalassemia is a hereditary form of anemia that affects the synthesis of hemoglobin. The management of therapy in patients with b-thalassemia major which patients should receive continuous blood transfusions and increased iron absorption from the digestive tract causes excess iron in the body. This will lead to an increase of free iron level that triggers Radical Oxygen Species (ROS). Increased level of ROS can initiate lipid peroxidation which used as an indicator of oxidative stress in cells and tissues and produce reactive carbonyl, mainly malondialdehyde (MDA). Thus, MDA measurement is widely used as an indicator of lipid peroxidation. On the other hand, the risk of oxidative damage can be reduced by antioxidant, one of them is Vitamin E that is a fat-soluble vitamin with high potential antioxidant. The objective of this study was to analyze the effect of the dl-a-tocopherol (Vitamin E) administration on decrease of MDA serum level on pediatric patients with b-thalassemia major. This was a longitudinal observational study design for one group without comparison was conducted to examine the use of vitamin E to decreased MDA serum level on children patients with b-thalassemia major. The inclusion criteria were patients who rely on blood transfusions, patients who received only one type of iron chelating agents during the study period, the clinical condition is stable, agrees, and has completed the informed consent. In the course of the study of 21 patients there were variations in patient compliance in taking vitamin E tablet dosage 200 IU once-daily for one month: only 11 out of 21 patients consumed 30 tablets of vitamin E 200 IU (total dose of 6000 IU) in the 1-month study, and only data from those 11 samples will be analysed further. MDA serum level was measured pre- and post-administration of vitamin E and patient’s characteristics of subjects was obtained for additional information. Pre-administration of vitamin E, serum level of MDA was 1239.4 ± 502.55 ng/mL with a range of 216.95 to 2297.3 ng/mL, whereas in the group post administration of vitamin E, MDA serum level was 786.49 ± 704.88 ng/mL with a range of 6.5380 to 1958.6 ng/mL. In conclusion, there was no significant difference in MDA serum level in the group pre- and post- administration of vitamin E (p = 0.15).
Background: Hemophilia is a blood clotting disorder caused by a deficiency of blood clotting factors, namely FVIII deficiency (Hemophilia A) and FIX deficiency (Hemophilia B). World epidemiological data shows that the prevalence rate of Hemophilia A is 85-90% and Hemophilia B is 10-15%. Objective: To analyze the correlations between Hemophilia factor activity with APTT values and clinical manifestations of pediatric Hemophilia patients at RSUD Dr Soetomo Surabaya. Methods: This study used a retrospective observational design. Subjects were pediatric hemophilia patients who met the inclusion criteria from total sampling data for the period January 2018-December 2022 from patient medical records. Results: It is known that the majority of pediatric Hemophilia patients in the age range of 2-10 years, as many as 50%. Descriptive statistical values were expressed in (mean ± SD), including APTT value (69 ± 25), FVIII activity (4.6 ± 5.9), and FIX activity (12 ± 15). The most clinical manifestation of bleeding was joint bleeding (hemarthrosis). There were 23 patients with Hemophilia A and 3 patients with Hemophilia B. There was no significant relationship between Hemophilia factor activity and APTT values using the Chi-Square Test statistical test. There was also no significant relationship between hemophilia factor activity and manifestations of hemophilia using the Kruskal-Wallis correlation test. Conclusion: The results of this study indicate that there are no correlations between Factor VIII activity and Factor IX activity with APTT values or with clinical manifestations that occur in patients.
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.
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