Regular Transfusions Research Articles

Pediatric factor X111 deficiency with retroperitoneal hematoma: An anaesthesiologist’s challenge

A 12 year old girl, known case of factor X111 deficiency came with complains of pain abdomen and fever was diagnosed with retroperitoneal hematoma. Patient was on regular cryoprecipitate and FFP transfusions since childhood for factor deficiency. Initial blood investigations shows anemia and deranged coagulation profile. Ultrasonography shows retroperitoneal hematoma. Patient posted for surgical exploration. After pre operative evaluation and optimisation by blood products transfusion. Surgery was done under general anaesthesia with blood products reserved for emergency management of intra operative bleeding. Intra op was uneventful. Postoperatively there were no complications.

Psychosocial aspects in children with transfusion-dependent thalassemia

ABSTRACT Children with transfusion-dependent thalassemia experience stressful conditions related to chronic anemia, repeated blood transfusions, and iron chelation adherence. They are vulnerable to emotional and behavioral problems and psychosocial disturbances. An observational evaluation of psychosocial issues in children with TDTs aged 4–18 using Strengths and Difficulties Questionnaires (SDQ). Psychosocial part in children with TDTs often experiences difficulties in childhood under 10 years old, compared to adolescents (11–18 years) for emotional symptoms and conduct problems. Boys and girls had similar psychosocial problems regarding emotional symptoms, conduct problems, hyperactivity-inatttention, peer relationship problems, and prosocial behavior. In a quarter of cases, they had emotional symptoms, conduct, and hyperactivity-inattention issues, especially in younger children. Interestingly, younger children have more problems than adolescence in peer relations even though they are still under strict parental supervision. Difficulties in emotion, conduct, and hyperactivity would affect their relationship life. Children may try to be accepted by their social environment (prosocial) even though they realize that there are problems in their emotional life and peer relationships. Regular screening of the psychosocial aspects should be followed by counseling because regular transfusion and iron chelation are necessary for long-term management.

Evaluation of a prophylactic transfusion program on obstetric outcomes in pregnant women with sickle cell disease: A single centre retrospective cohort study

ObjectiveTo evaluate the effects of a prophylactic transfusion program (TP) on obstetric and perinatal outcomes in pregnant women with sickle cell disease (SCD). MethodsThis retrospective cohort study included all singleton pregnancies among women with SCD in a French university tertiary care center between 1 January 2004 and 31 December 2017. The TP group included patients selected according to the French guidelines who received regular red blood cell transfusions during pregnancy until delivery. The factors associated with TP indication [year of birth, SCD genotype, history of acute chest syndrome and delayed hemolysis transfusion reaction (DHTR) risk score] were taken into account in a propensity score. A composite obstetric adverse outcome was defined associating birth before 34 gestational weeks and/or pre-eclampsia and/or small for gestational age and/or abruption and/or stillbirth and/or maternal death and/or neonatal death. ResultsIn total, 246 pregnancies in 173 patients were analyzed. Twenty-two pregnancies with a history of DHTR were excluded. A higher frequency of TP was found before 2013 [119/148 (80.4%) vs 38/76 (50%); p < 0.001]. Rates of preterm birth before 34 gestational weeks (5.6% vs 19.7%; p = 0.001), vaso-occlusive crisis (36.5% vs. 61.8%; p < 0.001), and acute chest syndrome (6.1% vs. 14.5%; p = 0.04) during pregnancy were decreased significantly in the TP group. Among the groups with and without composite obstetric adverse outcomes, the frequency of TP was 52.6% and 74.7%, respectively [odds ratio (OR) 0.30, 95% confidence interval (CI) 0.09–1.02]. The multivariate analysis shows that the TP was associated with a significant reduction in the risk of composite obstetric adverse outcomes (OR 0.28, 95% CI 0.08–0.97; p = 0.04). ConclusionA red blood cell TP may have an independent protective effect on maternal and perinatal adverse outcomes during pregnancy in women with SCD.

Increased red blood cell alloimmunization rates in transfused aplastic anemia and myelofibrosis patients.

Red blood cell (RBC) alloimmunization (AI) is a well-known complication of RBC transfusions, which results in the formation of alloantibodies to non-self antigens on donor RBCs, putting patients at risk of transfusion-related complications. The rate of AI with RBC transfusions in the general hospitalized population is estimated to be 2%-3%. However, some patients who are deemed "transfusion-dependent" require regular transfusions of blood products due to persistently low cell counts, putting them at even greater risk of RBC AI and increased morbidity. However, few studies currently exist investigating RBC AI in some transfusion-dependent patient populations, e.g., aplastic anemia (AA) and myelofibrosis (MF). We conducted a 5-year retrospective review to investigate the prevalence of RBC AI, alloantibody incidence, and the number of RBC transfusions in AA and MF patients, who received RBC transfusions within our hospital system. During the study period, 64 AA and 93 MF patients received 1301 and 2766 RBC transfusions, respectively. Compared to the RBC AI rate in the generalized hospitalized patient population (1%-2%), patients with AA and MF had an increased rate of RBC AI incidence rate at 14.1% and 12.9%, respectively. Furthermore, patients with primary MF demonstrated an isolated increased RBC AI incidence rate of 13.3%. The most common alloantibodies produced were anti-E and anti-K. Within our institution, patients with AA and MF had increased incidence rates of RBC AI compared to the general hospitalized patient population and may benefit from an antigen-matched protocol to minimize AI-related complications.

Pattern and clinical correlates of renal iron deposition in adult beta-thalassemia major patients.

We evaluated pattern and clinical correlates of renal T2* measurements in adult β-thalassemia major (β-TM) patients. Ninety β-TM patients (48 females, 38.15 ± 7.94years), consecutively enrolled in the Extension-Myocardial Iron Overload in Thalassemia network, underwent T2* magnetic resonance imaging (MRI) for quantification of iron overload (IO) in kidneys, liver, pancreas, and heart. Ten (11.1%) patients showed renal IO (T2* < 31ms). Global kidney T2* values did not show a correlation with gender, age, splenectomy, regular transfusions or chelation starting age, pre-transfusion hemoglobin, and serum ferritin levels. Global kidney T2* values showed an inverse correlation with MRI liver iron concentration (LIC) values (R = - 0.349; p = 0.001) and a positive correlation with global pancreas T2* values (R = 0.212; p = 0.045). Frequency of renal IO was significantly higher in patients with cardiac IO than in patients without cardiac IO (50.0% vs. 6.3%; p = 0.001). A significant inverse association was detected between global kidneys T2* values and lactate dehydrogenase (LDH) (R = - 0.529; p < 0.0001). In multivariate regression analysis, MRI LIC and LDH were the strongest predictors of global kidney T2* values. A MRI LIC > 4.83mg/g dw predicted the presence of renal IO (sensitivity = 90.0%; specificity = 61.2%). Global kidney T2* values were inversely correlated with uric acid (R = - 0.269; p = 0.025). In conclusion, in adult β-TM patients, renal iron deposition is not common and is linked to both hemolysis and total body iron overload.

High Serum Ferritin Levels among Blood Transfused Thalassemic Patients Admitted to the Department of Paediatrics in a Tertiary Care Centre: A Descriptive Cross-Sectional Study.

Raised serum ferritin levels often indicate iron overload, but they are not specific as the levels are elevated in inflammatory disorders, liver diseases, alcohol excess, or malignancy. If regular transfusions are required for the patient with thalassemia, this doubles the rate of iron accumulation leading to earlier massive iron overload and iron-related damage. The aim of this study aimed to find out the prevalence of high serum ferritin levels among blood-transfused thalassemic patients admitted to the Department of Paediatrics in a tertiary care centre. A descriptive cross-sectional study was conducted at a tertiary care centre from 1 March 2022 to 31 December 2022. Ethical approval was taken from the Institutional Review Committee (Reference number: 078/79-017/HG). Children who were confirmed by haemoglobin electrophoresis on regular blood transfusion were included in the study. Those who did not gave consent were excluded from the study. Convenience sampling method was used. Point estimate and 90% Confidence Interval were calculated. Out of 53 cases, the prevalence of high serum ferritin level was seen in 46 (88.79%) (80.30-97.28, 95% Confidence Interval). Among 46, 34 (73.91%) had serum ferritin levels of more than 1000 to 2500 ng/ml whereas 12 (26.09%) had more than 25000 ng/ml. The prevalence of high serum ferritin levels among blood transfused thalassemic patients admitted to the Department of Paediatrics in a tertiary care centre was found to be higher than in other studies done in similar settings. blood transfusion; ferritin; thalassemia.

EE287 Healthcare Resource Utilization Among Patients with Transfusion-Dependent Beta-Thalassemia in England

Patients with transfusion-dependent β-thalassemia (TDT) have reduced or absent β-globin production and require regular red blood cell transfusions (RBCTs) for survival. This study aimed to describe the healthcare resource utilization (HCRU) associated with TDT in England.

EE58 Healthcare Resource Utilization Among Patients with Transfusion-Dependent Beta-Thalassemia in Italy

Patients with transfusion-dependent β-thalassemia (TDT) require regular red blood cell transfusions (RBCTs) for survival. This study sought to understand the healthcare resource utilization (HCRU) among patients with TDT in Italy.

Metformin as a Fetal Hemoglobin Inducer in Non-transfusion Dependent Thalassemia Patients

Non-transfusion dependent thalassemia (NTDT) refers to a group of thalassemic disorders who do not need regular transfusions for survival, however it may be needed in certain conditions. Metformin was reported as a potential fetal hemoglobin (HbF) inducing agent in vitro but its efficacy and safety in vivo was not fully studied. This is a prospective interventional study aimed at studying the effect of metformin on HbF change in NTDT. Methods: Patients with established diagnosis of NTDT were enrolled. They were receiving a stable fixed dose of Hydroxyurea over the last 3 months. Patients were divided into two groups: a group that received Metformin for 6 months (Metformin group) and a control group. Complete blood picture, reticulocytic count, hemoglobin electrophoresis, liver enzymes, bilirubin, kidney functions, LDH and random blood sugar were performed at onset, 3 and 6 months of the study. All adverse events were recorded. Results: Forty two patients aged 12–23 years were enrolled. Metformin intake over 6 months did not show any statistically significant difference in clinical or the laboratory variables of efficacy when compared to the control group apart from reticulocytic count which was higher in Metformin group throughout the study. Conclusion: Metformin intake, in addition to hydroxyurea, did not yield any extra benefit among patients with NTDT.

Red blood cell alloimmunizations in thalassaemia patients with regular transfusion in China: A systematic review and meta-analysis.

The development of red blood cell alloimmunization intensifies transfusion complication in thalassaemia patients. The purpose of this paper is to evaluate the existing evidence on the prevalence of erythrocyte alloimmunization in China by meta-analysis. We systematically searched cross-sectional studies regarding the alloimmunization of thalassaemia patients with regular blood transfusion in China from year 2000 to May 2021 in the Cochrane library, PubMed, EMBASE, Web of Science, and Chinese databases including CNKI, Wanfang Data, Vip and CBM. Data extraction and quality evaluation of the included studies were performed. Meta-analysis was performed using the DerSimonian and Laird random-effects models with inverse variance weighting. The presence of publication bias was tested by Egger's test, and the methodological quality of each included article was evaluated by the criteria specific to prevalence studies. A total of 1874 patients and 263 alloantibodies from 11 studies were identified and included in the meta-analysis. The proportion of alloantibodies against antigens belonging to the Rh, MNSs and Kidd systems were as high as 70.3%, 17.9%, and 6.5%, respectively. Meta-analysis showed that the overall prevalence of alloimmunization among transfusion-dependent thalassaemia patients in China is 11.4% (95%CI: 7.2%∼16.3%). The characteristics of red blood cell alloimmunization among thalassaemia patients with regular transfusion in China differ greatly from those in other countries. Therefore, transfusion strategies shall be actively adapted in line with thalassaemia patients in China to minimize the risk of alloimmunization.

Major histocompatibility complex (MHC) antigens polymorphism and alloimmunization study in thalassemia patients with febrile non-hemolytic transfusion reaction (FNHTR).

HLA alloimmunization is one of the most troublesome consequence of regular transfusion which is itself a mainstay measure to provide longevity to the thalassemia patients. Febrile non-hemolytic transfusion reaction (FNHTR) is one of the most common complication which might be related to the HLA alloimmunization. Here, we studied the HLA antigenic system and alloimmunization rate in the Iranian β-thalassemia patients who suffered from FNHTR compare to the β-thalassemia patients without FNHTR. Total of 60 β-thalassemia patients with FNHTR (case group) and 20 β-thalassemia patients without FNHTR (control group) randomly have been selected and enrolled in the study. All were tested for HLA-A and -B loci by PCR-SSP method and also for the presence of anti-lymphocyte antibodies by LIFT method. Comparisons between two groups were performed by Pearson's χ2 test. Totally, a significant predominance was noted for two HLA alleles, HLA-A*24 (P=0.029) and B*55 (P=0.034) which have higher prevalence in control group. Although no significant association was found between the presence of anti-leukocyte antibodies and the development of FNHTR, the HLA-A*32 (P=0.047) allele was considered as possible genetic markers in the susceptibility to the development of anti-leukocyte antibodies. Here some evidences about the possible role of HLA polymorphism in susceptibility to FNHTR are provided. Those results indicated that HLA-A*24 and HLA-B*55 might play protective role on inducing FNHTR in β-thalassemia patients. Further studies which investigate the allele level of HLA-I alongside with specific reactivity of HLA-I antibodies might reveal more deep data about these phenomena.

Bone Marrow Transplantation in Nonmalignant Haematological Diseases: What Have We Learned about Thalassemia?

Allogeneic stem cell transplantation remains the only therapy for congenital, severe haemoglobinopathies that is able to reverse the pathological phenotype. In the severe form of thalassemia, regular transfusions are needed early in life. This population of patients could benefit from allo-SCT. However, the great efficacy of transplantation must be counterbalanced by the mortality and morbidity related to the procedure. In this short review, we reviewed the most recent data in the field of transplantation in transfusion-dependent thalassemia (TDT), highlighting the factors that have a major impact on outcomes.

Management of luspatercept therapy in patients with transfusion-dependent β-thalassaemia.

Patients with transfusion-dependent β-thalassaemia require lifelong, regular red blood cell transfusions for survival; however, frequent blood transfusions are associated with an increased risk of iron overload, transfusion-transmitted disease and alloimmunization, as well as reduced quality of life. Luspatercept, an erythroid maturation agent that promotes late-stage erythroid maturation independently of erythropoietin, has demonstrated efficacy in reducing transfusion burden in patients with transfusion-dependent β-thalassaemia. In this review, we discuss treatment initiation, ongoing evaluation, dose adjustment and management of adverse events in transfusion-dependent patients with β-thalassaemia receiving luspatercept, and we provide guidance on how to determine whether patients are deriving clinical benefit.

Thalassaemia-A global view.

The thalassaemias are a group of genetic disorders of haemoglobin which are endemic in the tropics but are now found worldwide due to migration. Basic standard of care therapy includes regular transfusions to maintain a haemoglobin level of around 10 g/dL, together with iron chelation therapy to prevent iron overload. Novel therapies, bone marrow transplantation, and gene therapy are treatment options that are unavailable in many countries with stressed economies. This Wider Perspectives article presents the strategies for management of an adolescent refugee patient with beta thalassaemia, as it would be performed by expert haematologists in six countries: Italy, Lebanon, Oman, the Sudan, Thailand and the United States. The experienced clinicians in each country have adapted their practice according to the resources available, which vary greatly. Even in the current modern era, providing adequate transfusions and chelation is problematic in many countries. On the other hand, ensuring adherence to therapy, particularly during adolescence, is a similar challenge seen in all countries. The concluding section highlights the disparities in available therapies and puts the role of novel therapies into a societal context.

Transfusion-transmitted Malaria in a Pregnant Woman with Beta Thalassemia Minor: A Case Report

BACKGROUND: Malaria is an infectious disease that is caused by plasmodium parasites. Malaria is commonly spread by female anopheles mosquitoes carrying the plasmodium parasite, although it can also be transferred through blood transfusion. In the developing world, malaria frequently affects the most vulnerable, including small children and pregnant women, resulting in severe morbidity and mortality. CASE PRESENTATION: This case report presents a primigravida with beta-thalassemia who developed malaria during her pregnancy despite never having visited a malaria-endemic region. A 22-year-old primigravida in her 29th week of pregnancy presented to an outpatient clinic with a 1-week history of fever. Blood smears, both thick and thin, revealed Plasmodium malariae trophozoites, schizonts, and gametocytes. She was diagnosed with a mild form of beta-thalassemia and required monthly blood transfusions. We think the patient got malaria from a blood transfusion because she has never been to or lived where malaria is common. Infections transferred through blood transfusions should be prevented in thalassemia patients who require regular transfusions, particularly in vulnerable groups such as pregnant women. CONCLUSION: Transfusion-transmitted diseases can be prevented by screening donors who have a history of malaria and have traveled to endemic areas.

Electrophysiological properties and heart rate variability of patients with thalassemia major in Jakarta, Indonesia.

Beta thalassemia major (TM) is a common hereditary disease in Indonesia. Iron overload due to regular transfusion may induce myocardial iron deposition leading to electrophysiological dysfunction and functional disorders of the heart. Ventricular arrhythmia is one of the most common causes of sudden cardiac death in thalassemia patients. This cross-sectional study of 62 TM patients aged 10-32 years in Cipto Mangunkusumo General Hospital was done to assess their electrophysiological properties and heart rate variability, including 24- hour Holter monitoring, signal averaged electrocardiogram (SAECG) for detection of ventricular late potential (VLP), and determination of heart rate variability (HRV). We also assessed their 12-lead ECG parameters, such as P wave, QRS complex, QT/ QTc interval, QRS dispersion, and QT/ QTc dispersion. Iron overload was defined by T2-star magnetic resonance (MR-T2*) values of less than 20 ms or ferritin level greater than 2500 ng/mL. Subjects were grouped accordingly. There were significant differences of QTc dispersion (p = 0.026) and deceleration capacity (p = 0.007) between MR-T2* groups. Multivariate analysis showed an inverse correlation between QTc dispersion and MR-T2* values. There was a proportional correlation between heart rate deceleration capacity in the low MR-T2* group (p = 0.058) and the high ferritin group (p = 0.007). No VLPs were detectable in any patients. In conclusion, prolonged QTc dispersion and decreased heart rate deceleration capacity were significantly correlated with greater odds of iron overload among patients with Thalassemia major.

Prevalence and Risk Factors of Hepatitis B and Hepatitis C Virus Infections in Repeatedly Transfused Thalassemia Patients in a Tertiary Care Hospital

Background: Thalassemia patients who are conventionally treated by regular transfusion regimen are at a risk of acquiring transfusion transmitted infections, including hepatitis B and hepatitis C. Getting blood transfusion in different places makes them vulnerable to these blood borne infections. It is important to assess and update the prevalence of these infections along with their contributing factors for ensuring optimum preventive measures and further strengthening of the screening program. Objectives: To estimate the prevalence of hepatitis B and hepatitis C virus infections in repeatedly transfused thalassemia patients and to determine the risk factors for acquiring these infections. Methods: This cross-sectional study was carried out in the Department of Pediatric Hematology and Oncology, Bangladesh Shishu Hospital&amp; Institute, Dhaka, Bangladesh during the period July 2018 to December 2019. Total 73 thalassemia patients of 2 to 18 years were enrolled into the study following the inclusion and exclusion criteria. Demographic data and other related information were recorded in a standard data sheet. Hb%, SGPT, HBsAg, Anti-HBs titre, Anti-HCV were done in all patients. Collected data was checked and analyzed by computer based program SPSS version 26.0 for Windows. Results: Out of total 73 thalassemia patients, 44 were male and 29 were female. Mean age was 8.3±3.45 years where maximum number of patients belonged to 6-10 years. 2(2.7%) patients had positive HBsAg and 11(15.1%) had positive Anti-HCV antibody at the end of study. Prevalence of hepatitis B infection was associated with lack of immunization against it which was statistically significant (P&lt;0.001). Hepatitis C virus infection in thalassemia patients was significantly associated with increasing duration of transfusion (P=0.043), frequency of transfusion (P&lt;0.001) and elevated SGPT level (P&lt;0.001). Comparing Anti-HBs titre, it is also found that there was decreased level of immunity against ..........

A Comparative Study to Assess Parathyroid Hormone Status in Different Age Groups of b-Thalassaemia Major Individuals from Eastern India

Introduction: β-thalassaemia major, due to imbalance, missing or deficiency of β-globin chain synthesis pathway, is marked as a hereditary disorder. Homozygous state presents with severe anaemia. Regular blood transfusions and chelation therapy increase the life expectancy in thalassaemia patients. Due to recent advances in the treatment paradigm of β-thalassaemia major patients, there has been a significant increase in their lifespan but, due to the treatment related iron overload, endocrine defects like hypogonadism, diabetes mellitus, hypothyroidism and Hypoparathyroidism (HPT) have been seen to progress. Diverse studies state the occurrence of HPT to be from none upto almost 22.5% in patients. It has also been recognised that asymptomatic hypocalcaemia is much more common and can be overlooked unless precisely viewed. Aim: To estimate and compare the biochemical parameters related to bone turnover in regularly transfused thalassaemia patients in different age groups and to find out significant correlation among the biochemical markers. Materials and Methods: In this cross-sectional study conducted at Calcutta National Medical College, Kolkata, West Bengal, India, from January 2017 till January 2018, 100 β-thalassaemia major patients were enrolled. Serum Parathyroid Hormone (PTH), serum ferritin, ionic calcium (Ca2+), Alkaline Phosphatase (ALP), phosphorus (P) and 25-Hydroxy vitamin D (25-OHD) levels were estimated in these patients. Statistical evaluation was done by the Statistical Package for the Social Sciences (SPSS), version 12.0. Result was reported as mean±SD. Pearson correlation coefficient was used to determine the association of serum ferritin levels and parathormone levels. The p-value of &lt;0.05 was considered as statistically significant. Results: Out of 100 patients, 19 patients had HPT. The mean age was found to be 12.6 years on diagnosis, mean ionised calcium was 0.95 mmol/L, mean serum ferritin was 3045 μg/L (range 1209-10,000 µg/L) and mean serum phosphate was 1.88 mmol/L (range 1.50-2.73 mmol/L). Serum PTH values of 7.04 pg/mL was found to be low. Negative correlation serum PTH and ferritin levels were found. Significant higher values of mean serum ferritin (2789 μg/L) and lower values of parathormone (17.13 pg/mL) was found in the age group of 11-18 years as compared to the other age group of ≤10 years with serum ferritin and parathormones values being 1648 μg/L and 23.44 pg/mL, respectively. Conclusion: Regularly transfused β-thalassaemia major patients, inspite of receiving chelation therapy tend to develop altered calcium and vitamin D homeostasis once they enter second decade of life. Thereby, biochemical parameters related to bone profile which includes ionic calcium, vitamin D, phosphate and parathyroid hormones levels should stringently monitored once the patient crosses first decade of life to prevent any development of hypocalcaemia or overt HPT.

Severity scoring system to guide transfusion management in pediatric non-deletional HbH.

Hemoglobin (Hb) H is generally recognized as mild thalassemia, despite its actual phenotypic diversity. A disease severity scoring system to guide initiation of regular transfusion among severely affected pediatric patients has not previously been reported. Patients with HbH were classified into transfusion-dependent thalassemia (TDT) and non-transfusion-dependent thalassemia (NTDT) as a surrogate for disease severity. Alpha-globin genotypes and relevant clinical parameters associated with TDT were identified. Univariate and multiple logistic regression analyses were performed to yield the most suitable severity scoring system. From 246 patients with a median age of 14.3 (interquartile range 9.9-18.4) years initially enrolled into the study, the chance of having severe disease and developing TDT was remarkable only among patients with non-deletional HbH, for whom the scoring system was developed. Univariate and multiple logistic regression analyses resulted in three retained parameters associated with TDT, β-coefficients of which were used to develop the score. The final scoring system comprised age at diagnosis <2 years (score = 1), spleen size ≥3 cm (score = 1) and Hb at steady-state <7 (score = 4) or 7-8 g/dL (score = 3). A cutoff score ≥4 was associated with severe disease likely requiring regular transfusion (sensitivity 89.3%, specificity 81.4%), given regular transfusion resulted in maintained growth. The scoring system was validated in the second cohort of 77 non-deletional HbH, from which comparable sensitivity and specificity were obtained. The newly developed scoring system was practical and helpful to highlight severely affected pediatric non-deletional HbH patients with potential needs of regular transfusion. This can be used as a guide for optimal treatment and disease monitoring in the future.

Prospective case–control study on prevalence of anxiety disorders in chronically transfused hemophilia patients of age group 8–15 years and their parents

Background: Hemophilia is a hemoglobinopathy with significant impact on mental health of the patients as well as their families. Aims and Objectives: The aims and objectives are to assess the prevalence of anxiety disorders in chronically transfused hemophilia patients and their parents. Materials and Methods: 100 dyads of hemophilia patients of age group 8–15 years who are on regular transfusion of clotting factors and their parents were compared with 100 age and sex-matched controls who are pediatric patients from the outpatient department or ward but not suffering from hemophilia or any other chronic disorder. Pre-validated Hamilton Anxiety Score was used to evaluate anxiety level among the participants. Results: Participants suffering from hemophilia had anxiety prevalence of 32% while among the controls, anxiety prevalence of 16% was observed. Among parents of adolescents suffering from hemophilia, 45% showed anxiety as compared to 24% prevalence in the parents of control group. It was observed that anxiety was significantly more in elderly patients and in those patients who had lesser gap in transfusion of clotting factors. Conclusion: Hemophilia patients as well as their parents are at risk of developing anxiety disorders more than the normal counterparts. Therefore, identification followed by treatment at earlier stages warrants better prognosis of the patient.