Impact of blood transfusion on troponin I levels and outcomes after cardiac surgery: a cohort study

Objectives: Increased cardiac troponin I [TI] has been suggested to be a sensitive indicator of intraoperative myocardial injury. We investigated the association of transfusion on TI levels post-surgery and outcomes in patients undergoing elective cardiac surgeries

Methods: We conducted a retrospective review of 542 patients. Patients were divided into two groups based on TI levels at 24 hours [TI24] [> 6.5 microg/L vs.

Results: Red blood cell [RBC] transfusion was found to be associated with high TI levels [odds ratio [OR] = 2.33, p = 0.007, 95% confidence interval [CI]: 1.30–4.30]. A trend was observed when aortic cross-clamp time and preoperative ejection fraction were adjusted for [OR = 2.06, p = 0.080, 95% CI: 0.90–4.70]. An association was found between aortic cross-clamp time and high TI levels in the multivariable model [OR = 1.01, p = 0.028, 95% CI: 1.00–1.02]. Elevated TI levels was associated with higher mortality [OR = 4.15, p = 0.017, 95% CI: 1.29–13.08], renal failure [OR = 2.99, p = 0.004, 95% CI: 1.41-6.32], and increased length of stay in-hospital [OR = 4.50, p = 0.020, 95% CI: 0.69-8.30]

Conclusions: RBC transfusion is associated with increased TI24 post-cardiac surgery and worse outcomes, albeit a confounding effect cannot be excluded. Larger studies are required to confirm these findings

Immunophenotypic characteristics of T-acute lymphoblastic leukemia in Omani patients: a correlation with demographic factors

Objectives: To study and classify the immunophenotypic characteristics of Omani patients diagnosed with T-cell acute lymphoblastic leukemia [T-ALL] and to correlate the results with age and gender as well as biological factors [peripheral and bone marrow blast cells percentage]

Methods: Fifty cases from both genders and of all ages who fulfilled the inclusion criteria with a diagnosis of T-ALL were included in the study. Correlation of T-ALL subtypes with age, gender, and initial bone marrow and peripheral blood blast cells percentage was assessed using ANOVA

Results: Among the 50 T-ALL patients analyzed, 44 were male and six were female giving a male-to-female ratio of 7:1 [p = 0.007]. The average age of patients was 19.2 years with no significant differences in the three disease subtypes. No significant association was seen between the peripheral or bone marrow blast cell percentage and the differentiation stages of the neoplastic clone of T-ALL. All female patients were found to express an immature T-ALL phenotype

Conclusions: This study reports the subtypes of T-ALL in Oman for the first time. It is hoped that this will lead to a better understanding of the disease outcomes

Knowledge and attitudes towards clinical trial participation in Oman: a cross-sectional study

Objectives: Clinical trials are prospective studies on human subjects designed to answer various clinical questions. However, only a limited number of clinical trials have been conducted in Oman. This study aimed to assess the knowledge and attitudes of Omani patients and their relatives towards participating in clinical trials

Methods: This cross-sectional study was conducted between October 2015 and March 2016 among 174 patients and relatives attending the Haematology and Oncology Outpatient Clinics and Day Care Unit of the Sultan Qaboos University Hospital, Muscat, Oman. A self-administered questionnaire was designed to elicit participants' knowledge of and attitudes towards participation in clinical trials

Results: A total of 100 patients and relatives agreed to take part in the study [response rate: 57.5%]. The male-to-female ratio was 1:1.2. Only 31.3% of the studied population knew what clinical trials were and only 6.5% had themselves previously participated in a clinical trial. The majority agreed or strongly agreed that they would participate in clinical trials related to their own condition [59.2%]. Overall, 89.7% expected to be informed about potential clinical trials by their treating physicians

Conclusion: Omanis had a low level of knowledge of clinical trials and a very low rate of previous participation in such trials, despite a moderate level of interest. Patients should therefore be educated and informed of ongoing clinical trials in order to improve participation rates for clinical trials conducted in Oman

Impact of aberrant myeloid antigen expression on outcomes of patients with t-cell acute lymphoblastic leukemia

Objectives: To evaluate the impact of myeloid antigen expression on complete remission [CR], event-free survival [EFS], and overall survival [OS] in patients with T-cell acute lymphoblastic leukemia [T-ALL] treated with intensive chemotherapy

Methods: We retrospectively reviewed consecutive patients diagnosed with T-ALL and treated in Sultan Qaboos University Hospital and Royal Hospital in Oman between 2004 and 2010. The diagnosis of T-ALL was established using French-American-British classification or World Health Organization criteria. Patients were considered having myeloid antigen expression if they expressed CD13, CD33, or both [My+ and My-]

Results: Of the 39 patients, 38 were included in the study [25 patients with My- and median age of 18.4 years, 13 patients with My+ and median age of 22.0 years]. Median follow-up was 12 months. Thirty-two out of the total cohort were eligible for response-rate assessment. Twenty-nine patients [90.6%] achieved CR with one or two courses of chemotherapy with similar CR rates between the two groups [p = 0.880]. Twenty-five percent [5/20] of the patients with My- required two courses of induction, whereas 58.3% [7/12] of My+ required two courses of induction and the difference was statistically significant [p = 0.040]. In the multivariable analysis; age, gender, initial white blood cell count, central nervous system disease, and myeloid antigen expression were not statistically significant predictors of CR. The EFS and OS were similar between the My+ and My- groups p = 0.180 and p = 0.440, respectively

Conclusions: Patients with T-ALL with myeloid antigen expression need more courses of induction; however, rates of CR, EFS, and OS are not different from those without myeloid antigen expression. Larger prospective studies are required to confirm these findings

Therapeutic drug monitoring-guided dosing of busulfan differs from weight-based dosing in hematopoietic stem cell transplant patients

Busulfan [Bu]-based preparative regimens in hematopoietic stem cell transplantation are commonly used. Previous studies have shown that Bu at a fixed dose of 3.2 mg/kg/day [FBD] given intravenously decreases variability in drug pharmacokinetics and this decreases the dependency on therapeutic drug monitoring [TDM] of Bu. We compared the Bu dose given using TDM with the FBD of 3.2 mg/kg/day. Seventy-three patients with acute leukemia, myelodysplasia, chronic myeloid leukemia, thalassemia major, and sickle cell disease were included. The mean age at transplant was 15 years [range 2-55 years] with 57% adults. Indication for transplantation was leukemia/myelodysplastic syndrome in 46% of the patients, while the remaining 54% were transplanted for inherited blood disorders. We found that the median FBD was lower than the median TDM dose by 39 mg/day with a statistically significant difference [p < 0.001] even after adjusting for the weight [median total FBD of 349 mg, median TDM dose of 494 mg, p < 0.0001]. Age and underlying condition [malignant vs. nonmalignant] were the main factors affecting Bu clearance [p < 0.001 and p < 0.07, respectively]. TDM remains an important tool for the appropriate dosing of Bu in preparative regimens of hematopoietic stem cell transplantation, especially in populations with genetic admixture

Unusual indolent course of a chronic active epstein-barr virus-associated natural killer cell lymphoproliferative disorder

Natural killer [NK] cell lymphoproliferative disorders are uncommon and the Epstein-Barr virus [EBV] plays an important aetiological role in their pathogenesis. We report a 20-year-old male with a chronic active EBV infection associated with a NK cell lymphoproliferative disorder which had an unusual indolent course. He presented to the Sultan Qaboos University Hospital in Muscat, Oman, in December 2011 with a history of intermittent fever and coughing. Examinations revealed generalised lymphadenopathy, hepatosplenomegaly, leukocytosis, transaminitis, diffuse bilateral lung infiltrates and bone marrow lymphocyte involvement. A polymerase chain reaction [PCR] test revealed a high EBV viral load in the peripheral blood cells. The patient received a course of piperacillin-tazobactam for Klebsiella pneumoniae, but no active treatment for the lymphoproliferative disorder. However, his lymphocyte count, serum lactate dehydrogenase and liver enzymes dropped spontaneously. In addition, EBV PCR copies fluctuated and then decreased significantly. He remained clinically asymptomatic over the following four years

First implementation of transfusion consent policy in Oman

Objectives: transfusions are a common medical intervention. Discussion of the benefits, risks and alternatives with the patient is mandated by many legislations prior to planned transfusions. At the Sultan Qaboos University Hospital [SQUH], Muscat, Oman, a written transfusion consent policy was introduced in March 2014. This was the first time such a policy was implemented in Oman. This study therefore aimed to assess adherence to this policy among different specialties within SQUH

Methods: the medical records of patients who underwent elective transfusions between June and August 2014 were reviewed to assess the presence of transfusion consent forms. If present, the consent forms were examined for completeness of patient, physician and witness information

Results: in total, the records of 446 transfused patients [299 adult and 147 paediatric patients] were assessed. Haematology patients accounted for 50% of adult patients and 71% of paediatric patients. Consent was obtained for 75% of adult and 91% of paediatric patients. The highest adherence rate was observed among adult and paediatric haematology specialists [95% and 97%, respectively]. Consent forms were correctly filled out with all details provided for 51% and 52% of adult and paediatric patients, respectively. Among inadequately completed forms, the most common error was a lack of witness details [20-25%]

Conclusion: in most cases, the pre-transfusion consent policy was successfully adhered to at SQUH. However, further work is required to ensure full compliance with the consent procedure within different specialties. Implementation of transfusion consent in other hospitals in the country is recommended

Reproducibility of manual platelet estimation following automated low platelet counts

Objectives: Manual platelet estimation is one of the methods used when automated platelet estimates are very low. However, the reproducibility of manual platelet estimation has not been adequately studied. We sought to assess the reproducibility of manual platelet estimation following automated low platelet counts and to evaluate the impact of the level of experience of the person counting on the reproducibility of manual platelet estimates

Methods: In this cross-sectional study, peripheral blood films of patients with platelet counts less than 100 × 109 /L were retrieved and given to four raters to perform manual platelet estimation independently using a predefined method [average of platelet counts in 10 fields using 100× objective multiplied by 20]. Data were analyzed using intraclass correlation coefficient [ICC] as a method of reproducibility assessment

Results: The ICC across the four raters was 0.840, indicating excellent agreement. The median difference of the two most experienced raters was 0 [range: -64 to 78]. The level of platelet estimate by the least-experienced rater predicted the disagreement [p = 0.037]. When assessing the difference between pairs of raters, there was no significant difference in the ICC [p = 0.420]

Conclusions: The agreement between different raters using manual platelet estimation was excellent. Further confirmation is necessary, with a prospective study using a gold standard method of platelet counts

Rituximab leads to long remissions in patients with chronic immune thrombocytopenia

To assess the response rate and duration of response in patients with chronic immune thrombocytopenia [ITP] receiving rituximab. We retrospectively analyzed 32 consecutive patients with chronic ITP who were treated in two tertiary centers in Oman. Response assessment was based on the American Society of Hematology criteria. Nineteen patients [59%] had an initial response. However, six of the 19 patients lost their response leaving 13 patients with long-lasting remissions. The median age at diagnosis was 25 years [range 14-58]. The median time from diagnosis to rituximab therapy was 21 months. The median follow-up after starting rituximab was 26 months. The overall cumulative response rate was 59% [complete response 44%, partial response 15%] and the median time to respond was 30 days with a response rate of 44% at four weeks. In all responders, the cumulative rate of loss of response was 32% with a median time to lose response of 54 months. The use of rituximab in ITP achieves high response rate and long remission duration. Our study was limited by the small sample size and further larger prospective studies are recommended

Methemoglobinemia in an elderly patient with glucose-6-phosphate dehydrogenase deficiency: a case report

Glucose-6-phosphate dehydrogenase [G6PD] deficiency is an X-linked genetic disorder characterized by low levels of the G6PD enzyme. It is present worldwide but with more prevalence in the Middle East and the Mediterranean areas. We report a case of severe hemolysis due to G6PD deficiency manifesting as methemoglobinemia in a 70 year old Omani male never known to have any previous hemolytic episodes or previously diagnosed of G6PD deficiency.

Knowledge and attitudes of Oman medical specialty board residents towards evidence-based medicine

This study aims to evaluate the knowledge and attitudes of Oman Medical Specialty Board [OMSB] residents towards Evidence-Based Medicine [EBM]. This cross sectional study was conducted on all OMSB residents through a self-administered online questionnaire between October 2012 and March 2013. An electronic survey was designed to identify and determine residents' knowledge and attitudes toward the use of EBM. The survey was completed by 93 [21%] OMSB residents, 76 [82%] of whom took part in continuing education courses and 50 [54%] belonged to professional practice-oriented organizations. On average, the residents were reportedly involved in patient care for approximately 70% [Standard Deviation [SD] 17%] of their time, while 14% [SD 12%] participated in research activities. The results showed that 53 respondents [57%] were competent users of medical search engines compared to 23 residents [25%] who rated their skills as neutral. Sixteen percent of the respondents strongly agreed and 46% only agreed that the facility supports the use of current research in practice. Fourteen percent strongly agreed and fifty-three percent only agreed that the foundation of EBM is part of OMSB academic preparation. On the other hand, 17% of the respondents thought that insufficient time is always a barrier against EBM, while another 27% perceived insufficient time as a usual barrier. The lack of information resources was reported to always be a barrier in 11% of the respondents while 32% thought that it usually acts as a barrier. Time constraints and skills in EBM were found to be the two major obstacles. This study was, however, limited by the low response rate of the survey; thus larger studies with a previously validated questionnaire should be conducted in the future

Recognition, investigation and management of acute transfusion reactions: consensus guidelines for Oman

The recognition and management of transfusion reactions [TRs] are critical to ensure patient safety during and after a blood transfusion. Transfusion reactions are classified into acute transfusion reactions [ATRs] or delayed transfusion reactions, and each category includes different subtypes. Different ATRs share common signs and symptoms which can make categorisation difficult at the beginning of the reaction. Moreover, TRs are often under-recognised and under-reported. To ensure uniform practice and safety, it is necessary to implement a national haemovigilance system and a set of national guidelines establishing policies for blood transfusion and for the detection and management of TRs. In Oman, there are currently no local TR guidelines to guide physicians and hospital blood banks. This paper summarises the available literature and provides consensus guidelines to be used in the recognition, management and reporting of ATRs

Perinatal outcome in pregnancies with extreme preterm premature rupture of membranes [Mid-Trimester PROM]

Preterm premature rupture of membranes [PPROM] is defined as the rupture of fetal membranes before 37 weeks. Extreme PPROM occurs before 26 weeks' gestation and can result in perinatal morbidity and mortality. The aim of this study was to study the perinatal outcomes of mothers with extreme PPROM. A retrospective cohort study of 44 consecutive pregnant women, presenting with PPROM before 26 weeks' gestation, was conducted from January 2006 to December 2011 at Sultan Qaboos University Hospital, Oman. Maternal and neonatal information was collected from medical records, and delivery and neonatal unit registries. Women with PPROM presenting after 26 weeks' gestation, those with multiple gestations, or other types of preterm deliveries were excluded from the study. Of the 44 preterm infants admitted to the Neonatal Intensive Care Unit, 24 [55%] survived, 7 [16%] died within 24 hours of birth, 9 [20%] were miscarried, and 4 [9%] were stillbirths. Neonatal sepsis and pulmonary hypoplasia were the major causes of death. Neonatal complications among the surviving infants included prematurity in 11 [46%], respiratory distress syndrome in 19 [79%], sepsis in 12 [50%], and low birth weight in 11 [46%]. The neonatal survival rate was significantly associated with the gestational age at delivery but not with the gestational age upon rupture of membranes. Extreme PPROM was associated with adverse perinatal outcomes. The results of this study will help obstetricians and neonatologists in counseling couples experiencing PPROM. Future studies of long-term neonatal morbidity should have larger sample sizes and include more hospitals

Extreme preterm premature rupture of membranes: risk factors and feto maternal outcomes

Preterm premature rupture of membranes [PPROM] is defined as a rupture of the amniotic membranes occurring before 37 weeks of gestation and before the onset of labor. Extreme PPROM occurs prior to 26 weeks gestation and contributes to an increased risk of prematurity, leading to maternal and fetal complications. This study aims to estimate the risk factors associated with various maternal complications and to determine the worst outcomes in Omani females with extreme PPROM. A retrospective cohort study was conducted on 44 women with extreme PPROM, who delivered at Sultan Qaboos University Hospital [SQUH] from January 2006 to December 2011. Women with incomplete information, multiple gestations, or a preterm delivery resulting from medical intervention, as well as women who delivered elsewhere were excluded from the study. Forty-four women with extreme PPROM were included in our study. The results revealed the most important risk factor to be history of infection, which was noted in 24 study participants. The mean maternal age was 30 years. The mean gestational age at PPROM and at delivery were 20.7 +/- 3.2 [range: 16-26 weeks] and 29.7 +/- 7.6 weeks [range: 17-40 weeks], respectively. The maternal complications observed in this study included; infection which was seen in 20 [45%] patients, antepartum hemorrhage in 11 [25%] patients, and cesarean section which was required in 12 [27%] patients. There was no significant association between risk factors such as gestational age at delivery, parity, maternal age at PPROM, or maternal Body Mass Index [BMI] and cesarean section rate. Infection played a major role, both as a risk factor and in causing extreme PPROM, which in turn increased in 12 patients [27%]. In the multivariable model for predicting the need for cesarean section [gestational age at delivery, parity, maternal age at PPROM in years and maternal BMI], none of the factors were statistically significant. Overall, concurrent infection rate was high among patients presenting with extreme PPROM. None of the baseline maternal factors predicted the need for cesarean section. This is likely due to the small sample size; hence, larger prospective studies are needed to confirm these findings

Side effects of deferasirox iron chelation in patients with beta thalassemia major or intermedia

Chelating agents remain the mainstay in reducing the iron burden and extending patient survival in homozygous beta-thalassemia but adverse and toxic effects may increase with the institution and long term use of this essential therapy. This study aimed to estimate the incidence of deferasirox [DFX] side effects in patients with thalassemia major or intermedia. A retrospective study of 72 patients [mean age: 20.3 +/- 0.9 yrs; 36 male, 36 female] with thalassemia major or intermedia treated at Sultan Qaboos University Hospital, Oman, was performed to assess the incidence of side effects related to deferasirox over a mean of 16.7 month follow-up period. Six patients experienced rashes and 6 had gastro-intestinal upset. DFX was discontinued in 18 patients for the following reasons: persistent progressive rise[s] in serum creatinine [7 patients; 40% mean serum creatinine rise from baseline], feeling unwell [2], severe diarrhea [1], pregnancy [1], death unrelated to chelator [2] and rise in serum transaminases [2]. Three patients were reverted to desferoxamine and deferiprone combination therapy as DFX was no longer biochemically effective after 18 months of therapy. There was no correlation between baseline serum ferritin and serum creatinine or a rise in serum creatinine. Cardiac MRI T2* did not change with DFX therapy. However, there was an improvement in liver MRI T2* [p=0.013]. Renal side effects related to deferasirox appear to be higher than those reported in published clinical trials. Further larger studies are required to confirm these findings

Perinatal outcome of monochorionic in comparison to dichorionic twin pregnancies

The aim of this study is to compare the neonatal outcomes of monochorionic and dichorionic twin pregnancies. A retrospective cohort study involving 51 twin pregnancies followed and delivered at Sultan Qaboos University Hospital was conducted between January 2006 and December 2011. Thirty six [71%] pregnancies were dichorionic diamniotic [DCDA], 14 [27%] were monochorionic diamniotic [MCDA], and one [2%] was monochorionic monoamniotic [MCMA]. The antepartum complications noted in the 15 monochorionic twins were discordant fetal growth in 2 [14%] cases, low birth weight in 11 [73%] babies, pre-eclampsia in three mothers [21%] and twin to twin transfusion syndrome in four [29%] cases. Fetal respiratory distress affected eight [57%] of the pregnancies. Six [40%] twin sets were delivered before 30 weeks, 4 [27%] sets at 31 to 32 weeks, 2 [13%] sets at 34-35 weeks, 2 [13%] sets at 36-37 weeks, and 1 [7%] at 37-38 weeks. Fifteen mothers delivered 16 live infants, 9 [30%] stillbirths and 5 [17%] died after birth. Most neonatal deaths were due to neonatal sepsis and pulmonary hypoplasia. Dichorioinic twins, [DC] morbidity was seen in 11% and 40% for monochoroinic twins [MC]. Mortality rate was 17% for DC and 47% for MC twins. Perinatal morbidity and mortality remain high among monochorionic twins. This is likely due to frequent twin-to-twin transfusion syndrome, prematurity, fetal growth restriction and intrauterine fetal death. Improved fetal and neonatal management may result in improved outcomes

Genital ulcer development in patients with acute promyelocytic leukaemia treated with all-trans retinoic acid: a case series

We report here four cases of genital ulcers that developed after the administration of all-trans retinoic acid [ATRA] for the treatment of acute promyelocytic leukemia [APL]. Between October 2007 and March 2010, three males and one female [age range 19-35 years] were identified to have genital ulcers after being prescribed all-trans retinoic acid [ATRA] as a part of chemotherapy for APL. This is the first series of cases describing genital ulcers, as a unique and rare complication of ATRA used for treatment of APL in these patients, with no other cause identified. Following temporary cessation of ATRA for a few days in these three cases, improvement of the ulcers was noted