Murtadha Al-Khabori

Side effects of Deferasirox Iron Chelation in Patients with Beta Thalassemia Major or Intermedia

OBJECTIVES 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. METHODS 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. RESULTS 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). CONCLUSION 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.

Improved survival using an intensive, pediatric-based chemotherapy regimen in adults with T-cell acute lymphoblastic leukemia

All patients with newly diagnosed T-cell acute lymphoblastic leukemia (T-ALL) and treated over a 17-year period at a single institution were retrospectively analyzed. From 1990 to 2000, 40 patients were treated with a variety of adult-based ALL regimens. From 2000 to 2007, a pediatric-based protocol, DFCI (Dana Farber Cancer Institute), was used as the standard regimen for all patients (n = 32). The two groups (DFCI and non-DFCI) had comparable baseline characteristics. Complete response rates were not significantly different between the DFCI- and non-DFCI-treated groups. The 3-year relapse free survival (RFS) and overall survival (OS) were significantly higher in the DFCI-treated group (p < 0.0001 and p = 0.0003, respectively). On multivariate analysis, the treatment group (DFCI vs. non-DFCI) was the major prognostic factor influencing both RFS and OS. The results provide evidence supporting the superior efficacy of asparaginase-intensive pediatric-based regimens for adults with T-ALL.

T2* MRI in Regularly Transfused Children with Thalassemia Intermedia: Serum Ferritin Does Not Reflect Liver Iron Stores

Nontransfused patients with thalassemia intermedia (TI) accumulate iron due to increased gastrointestinal absorption of iron. Recent studies using T2* MRI revealed that serum ferritin does not reflect the severity of iron overload in nontransfused TI patients. We evaluated the iron overload status in TI children on monthly transfusion. Based on serum ferritin levels, 11 such patients (mean age 13.18 ± 4.09 years), were classified into two groups, group 1 (six patients) and group 2 (five patients) with serum ferritin levels below and above 1000 ng/mL, respectively. T2* MRI assessments were done for evaluation of hepatic and cardiac iron status. Group 1 and group 2 had mean serum ferritin levels of 817.300 ± 244.690 ng/mL and 1983.80 ± 662.862 ng/mL, respectively (P = .003). T2* MRI showed comparable moderate to severe hepatic iron overload status in both. None of the patients had myocardial iron deposition. We conclude that serum ferritin does not reflect the hepatic iron overload status in our patients with TI on regular transfusion.

Validation of a non-invasive pulse CO-oximetry based hemoglobin estimation in normal blood donors

INTRODUCTION Non-invasive hemoglobin estimation may increase the recruitment of blood donors. CO-oximetry hemoglobin estimation is a non-invasive method used to estimate the hemoglobin level. The primary objective of this study is to validate the pulse CO-oximetry based hemoglobin estimation in normal blood donors. METHODS We conducted a prospective observational study on 106 in a tertiary care hospital blood bank over a period of 4 weeks. We performed a Spot Hemoglobin concentration (Sp Hb) using Masimo Pronto-7 Pulse CO-oximetry, and compared it to a venous sample Hb concentration (Reference Hemoglobin; Ref Hb) measured using Abbott CELL-DYN Sapphire hematology analyzer. Age, gender, weight, height, blood pressure and reference hemoglobin were used in the multivariable linear regression model of the difference in measurement. RESULTS Total of 106 donors (98 males, 8 females) were enrolled with a mean age and Ref Hb of 27 years (SD 6.2; 18-49) and 14.2 g/dL (SD 1.2; 11.5-17) respectively. The mean Sp Hb was 14.4 g/dL (SD 1.2;11.3-16.7). The mean difference between the Sp Hb and Ref Hb was 0.2 g/dL (SD 1.2;-4.5 to 3) with a correlation coefficient of 0.46 (R(2)=21%). In the multivariable model, height (p=0.015) and Hb level (p<0.001) were statistically significant predictors. A strong correlation was found between the two CO-oximetry Hb measurements (coefficient 0.78, R(2)=60%). CONCLUSIONS Our study validated the use of the CO-oximetry in blood donors. Larger prospective studies are needed to confirm our results.

Transfusion indication predictive score: a proposed risk stratification score for perioperative red blood cell transfusion in cardiac surgery

Red blood cell transfusion is known to be associated with increased morbidity and mortality in cardiac surgery. This study was performed to derive a score to predict that risk in our patients.

The pneumatic tube system does not affect complete blood count results; a validation study at a tertiary care hospital

Effect of the pneumatic tube system (PTS) on sample quality is controversial. Herein we aim at evaluating the impact of sample transportation via the PTS on complete blood count (CBC) results.

Successful management of severe hemolytic disease of the fetus due to anti-Jsb using intrauterine transfusions with serial maternal blood donations: a case report and a review of the literature

The management of pregnant women with anti‐Jsb is challenging due to the paucity of antigen‐negative blood for fetal and neonatal transfusion.

Discriminatory power of the intraoperative cell salvage use in the prediction of platelet and plasma transfusion in patients undergoing cardiac surgery

INTRODUCTION We aim to assess the discrimination of transfused salvaged blood in predicting perioperative platelet and plasma transfusion. METHODS Retrospective review was performed on all patients undergoing cardiac surgery with cell saver (CS) support. The area under the receiver operating characteristics curve was calculated. RESULTS The discrimination achieved by transfused CS volumes in predicting perioperative platelet and plasma transfusion was poor (AUC 0.642 and 0.613 respectively). None of the covariates included (preoperative platelets, cardiopulmonary bypass use and time, aortic cross clamp time and use of aspirin or clopidogrel within 7 days of surgery) were statistically significant predictors. CONCLUSION Volumes of transfused CS blood have poor discrimination in predicting platelet and plasma transfusion.

Perinatal outcome of monochorionic in comparison to dichorionic twin pregnancies.

OBJECTIVE The aim of this study is to compare the neonatal outcomes of monochorionic and dichorionic twin pregnancies. METHODS A retrospective cohort study involving 51 twin pregnancies followed and delivered at Sultan Qaboos University Hospital was conducted between January 2006 and December 2011. RESULT 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. CONCLUSIONS 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.

Safety of stem cell mobilization in donors with sickle cell trait

High WBC plays a major role in the pathogenesis of many of the complications seen in patients with sickle cell disease (SCD).1 Individuals with Sickle cell trait may develop SCD-related complications in conditions of severe stress.2 G-CSF increases the WBC and may contribute to some of the complications in individuals with sickle cell trait when used in stem cell mobilization. To the best of our knowledge, only one small study3 assessed the safety of the mobilization of stem cells in individuals with sickle cell trait. Herein, we aimed to compare mobilization adverse events between donors with and without sickle cell trait.