Transfusion in haemoglobinopathies: review and recommendations for local blood banks and transfusion services in Oman

Sickle cell disease and homozygous beta-thalassaemia are common haemoglobinopathies in Oman, with many implications for local healthcare services. The transfusions of such patients take place in many hospitals throughout the country. Indications for blood transfusions require local recommendations and guidelines to ensure standardised levels of care. This article summarises existing transfusion guidelines for this group of patients and provides recommendations for blood banks and transfusion services in Oman. This information is especially pertinent to medical professionals and policy-makers developing required services for the standardised transfusion support of these patients

Single breath-hold physiotherapy technique effective tool for T2[asterisk] magnetic resonance imaging in young patients with thalassaemia major

Magnetic resonance imaging using T2[asterisk] [MRI T2[asterisk]] is a highly sensitive and non-invasive technique for the detection of tissue iron load. Although the single breath-hold multi-echo T2[asterisk] technique has been available at the Sultan Qaboos University Hospital [SQUH], Muscat, Oman, since 2006, it could not be performed on younger patients due to their inability to hold their breath after expiration. This study was carried out between May 2007 and May 2015 and assessed 50 SQUH thalassaemic patients aged 7-17 years old. Seven of these patients underwent baseline and one-year follow-up MRI T2[asterisk] scans before receiving physiotherapy training. Subsequently, all patients were trained by a physiotherapist to hold their breath for approximately 15-20 seconds at the end of expiration before undergoing baseline and one-year follow-up MRI T2[asterisk] scans. Failure rates for the pre- and post-training groups were 6.0% and 42.8%, respectively. These results indicate that the training of thalassaemic patients in breathhold techniques is beneficial and increases rates of compliance for MRI T2[asterisk] scans

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

Prevalence of hepatitis C among multi-transfused thalassaemic patients in Oman single centre experience

Regular blood transfusions are essential for patients with thalassaemia major. However, infections with hepatotropic viruses remain a major concern. The objective of this study was to evaluate the prevalence and characteristics of hepatitis C viral [HCV] infection among patients with homozygous beta thalassaemia in a single centre in Oman. A retrospective chart review of 200 patients treated at the Thalassemia Unit of Sultan Qaboos University Hospital [SQUH] in Muscat, Oman, between August 1991 and December 2011 was performed. Relevant demographic and clinical characteristics were collected, including age, gender, HCV status and the presence of endocrinopathies. A total of 81 patients [41%] were found to be anti-HCV-antibody [anti-HCV]-positive. HCV ribonucleic acid tests were performed on 65 anti-HCV-positive patients and were positive among 33 [51%]; the remaining 16 patients died before these tests were available. Anti-HCV-positive patients were significantly older than anti-HCV-negative patients [P <0.001] and were more likely to be diabetic than anti-HCV-negative patients [27% versus 8%; P <0.001]. A total of 100 patients had been transfused before they were transferred to SQUH in 1991; of these, 70 [70%] were anti-HCV-positive. Only 11 [11.5%] of the 96 patients who were seronegative in 1991, or who were transfused later, became seropositive. It is likely that the high prevalence of HCV among multi-transfused thalassaemic patients in Oman is due to blood transfusions dating from before the implementation of HCV screening in 1991 as the risk of HCV-associated transfusions has significantly reduced since then. Additionally, results showed that anti-HCV-positive patients were more likely to be diabetic than anti-HCV-negative patients

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

Pregnancy outcomes in women with homozygous beta thalassaemia: a single-centre experience from Oman

Pregnancy in women with homozygous beta thalassaemia [HBT] carries a high risk to both the mother and fetus. The aim of this study was to investigate pregnancy outcomes among this group at a single tertiary centre. This retrospective descriptive study was conducted between January 2006 and December 2012 on all women with HBT who received prenatal care and subsequently delivered at Sultan Qaboos University Hospital, Muscat, Oman. Women who delivered elsewhere and women with the beta thalassaemia trait were excluded. Ten women with HBT were studied with a total of 15 pregnancies and 14 live births. The mean maternal age +/- standard deviation [SD] was 27.9 +/- 3.7 years, with a range of 24-35 years. There were 14 spontaneous pregnancies and one pregnancy following hormone treatment. Eight women had been on chelation therapy before pregnancy, one of whom needed chelation during late pregnancy. Of the pregnancies, 93% had a successful outcome with a mean +/- SD gestational age at delivery of 38.6 +/- 0.9 weeks, with a range of 37-40 weeks. Eight babies [57%] were delivered by Caesarean section. The mean +/- SD birth weight was 2.6 +/- 0.2 kg, with a range of 1.9-3.0 kg. Three babies [21%] were born with low birth weights. Pregnancy is safe and usually has a favourable outcome in patients with HBT, provided that a multidisciplinary team is available. This is the first study of Omani patients with HBT whose pregnancies have resulted in a successful outcome

Non-invasive haemoglobin estimation in patients with thalassaemia major

This study aimed to validate pulse CO-oximetry-based haemoglobin [Hb] estimation in children and adults with thalassaemia major [TM] and to determine the impact of different baseline variables on the accuracy of the estimation. This observational study was conducted over a five-week period from March to April 2012. A total of 108 patients with TM attending the daycare thalassaemia centre of a tertiary care hospital in Muscat, Oman, were enrolled. Spot [Sp] Hb measurements were estimated using a Pronto-7 [Registered Sign] pulse CO-oximetry device [Masimo Corp., Irvine, California, USA]. These were compared to venous samples of Hb using the CELL-DYN Sapphire Hematology Analyzer [Abbott Diagnostics, Abbott Park, Illinois, USA] to determine the reference [Ref] Hb levels. A multivariable linear regression model was used to assess the impact of baseline variables such as age, gender, weight, height, Ref Hb and blood pressure on the Hb estimations. Of the 108 enrolled patients, there were 54 males and 54 females with a mean age of 21.6 years [standard deviation [SD] = 7.3 years; range: 2.5-38 years]. The mean Ref Hb and Sp Hb were 9.4 g/dL [SD - 0.9 g/dL; range: 7.5-12.3 g/dL] and 11.1 g/dL [SD = 1.2 g/dL; range: 7.5-14.7 g/dL], respectively. The coefficient of determination [R[2]] was 21% with a mean difference of 1.7 g/dL [SD = 1.1 g/dL; range: -0.9-4.3 g/dL]. In the multivariable model, the Ref Hb level [P = 0.001] was the only statistically significant predictor. The Pronto-7 [Registered Sign] pulse CO-oximetry device was found to overestimate Hb levels in patients with TM and therefore cannot be recommended. Further larger studies are needed to confirm these results

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

Rapid iron loading in heart and liver in a patient with transfusion dependent thalassaemia after brief poor compliance with iron chelation therapy

Iron loading in patients with transfusion dependent thalassaemia is considered to occur primarily in the liver and, once the liver becomes saturated, other organs begin loading. We report here a splenectomised male patient who was treated for hepatitis C virus infection. Prior to starting antiviral therapy, his serum ferritin was maintained below 500 ng/ml with deferiprone monotherapy; cardiac T2[*] by magnetic resonance imaging was 48.8ms and hepatic T2[*] was 19.5ms. After twelve months of antiviral treatment during which time he was very poorly compliant with his deferoxamine chelation therapy, his ferritin had risen to 3820 ng/ml and cardiac and hepatic T2[*] findings were 12.7 ms and 14.5 ms respectively, indicating increased iron loading in both organs, but particularly in the heart. Fifteen months after recommencing combination chelation, his ferritin was 95 ng/ml and cardiac and hepatic T2[*] were 27.5 and 28.4ms respectively, indicating complete clearance of iron load in both organs. This case demonstrates that iron overload can develop rapidly and in some cases there is relatively rapid iron loading in the heart as compared to the liver

Agranulocyosis in beta thalassemia major patients treated with oral iron chelating agent [deferiprone]

Deferiprone is an oral chelating agent that has been recently shown to reduce cardiac siderosis, but is also known to be associated with serious side effects like agranulocytosis which can be fatal. This report is a single centre experience of 5 cases with severe agranulocytosis in amongst 144 patients [3.47%] of thalassemia major on combined chelation therapy with subcutaneous desferrioxamine and oral deferiprone which is much higher than the previous reports

Diagnosis of beta-thalassaemia carriers in the Sultanate of Oman

Haemoglobinopathies are a major cause of morbidity in the Sultanate of Oman and premarital screening is being encouragedin order to reduce the number of affected births. The identification of beta-thalassaemia carrier status is an essential prerequisite of any screening programme. However, the level of Haemoglobin [Hb] A[2], which is used to detect beta-thalassaemia carriers, can be affected by other factors including iron deficiency, concurrent alpha thalassaemia and the type of DNA mutation present. The following study was undertaken to ascertain if the Hb A[2] level is an appropriate tool for the identification of beta-thalassaemia carriers in the Omani population. Hb A[2] was measured by high performance liquid chromatography [HPLC] in 160 obligate carriers of beta-thalassaemia. 158 subjects had Hb A[2] levels above 3.5% indicating beta-thalassaemia trait. Two subjects had slightly lower levels and were found to be iron deficient. After therapy both these subjects' Hb A[2] levels increased to above 3.5%. In the absence of iron deficiency, Hb A[2] is an accurate marker for the presence of beta-thalassaemia trait in the Sultanate of Oman

Fluctuating antibody response in a cohort of hepatitis C patients

This project was designed to longitudinally study persons who had antibodies to hepatitis C virus [HCV] to characterise the serologic course of infection. The subjects were 149 multitransfused patients [141 with thalassaemia major, 3 with thalassaemia intermedia, and 5 with sickle cell anaemia] who had been regularly followed up for 3 to 7 years. Sequential serum samples obtained semi-annually between January 1994 and January 2001 were tested, prospectively, by second or third generation HCV enzyme-linked immunosorbent assay [ELISA], followed by confirmatory recombinant immunoblot assay [RIBA-2 or RIBA-3]. Of the 149 patients, 90 did not seroconvert to HCV, whereas 59 had detectable antibodies. On the basis of RIBA results in these 59 patients, 24 [41%] had persistent high antibody levels to structural and non structural HCV antigens, 11 [19%] had persistent low antibody levels, 17 [29%] showed fluctuating antibody levels, and in 5 patients [8%] there was a total or a partial disappearance of specific antibodies [seroreversion], mainly anti-core antibodies. Two patients [3%] had antibody responses that did not fit into any of these four categories. In patients with fluctuating antibody levels, there were periods ranging from 6 months to 2 years when anti-HCV antibodies could not be detected. This study shows that the antibody response to HCV in patients who receive frequent blood transfusions is very variable. Individuals who exhibit intermittent seropositivity are a challenge to diagnosis