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

First cytogenetic profile of Omani patients with de novo myelodysplastic syndromes - Comparison with data from Asia, Africa, Europe and North and South America

Clonal cytogenetic abnormalities have been reported among 30-80% of patients with myelodysplastic syndromes [MDS]; however, 20-70% of patients with MDS show a normal karyotype that may nevertheless harbour a cryptic genetic alteration. Earlier reports have suggested that the distribution of specific chromosomal aberrations varies among Western and Asian countries, with geographical and ethnic differences in the frequency of specific chromosomal aberrations. This article compared the cytogenetic data of 36 adult Omani patients with MDS to previously reported data from other populations. Differences were noted between the percentages of clonal aberrations and the median age of Omani subjects at presentation in comparison to individuals of different ethnicities and from various geographical locations. To the best of the authors' knowledge, this is the first report to describe the cytogenetic data of patients with MDS from Oman

Impact of educational activities in reducing pre-analytical laboratory errors - a quality initiative

Objectives: Pre-analytic errors during diagnostic laboratory investigations can lead to increased patient morbidity and mortality. This study aimed to ascertain the effect of educational nursing activities on the incidence of pre-analytical errors resulting in non-conforming blood samples

Methods: This study was conducted between January 2008 and December 2015. All specimens received at the Haematology Laboratory of the Sultan Qaboos University Hospital, Muscat, Oman, during this period were prospectively collected and analysed. Similar data from 2007 were collected retrospectively and used as a baseline for comparison. Non-conforming samples were defined as either clotted samples, haemolysed samples, use of the wrong anticoagulant, insufficient quantities of blood collected, incorrect/lack of labelling on a sample or lack of delivery of a sample in spite of a sample request. From 2008 onwards, multiple educational training activities directed at the hospital nursing staff and nursing students primarily responsible for blood collection were implemented on a regular basis

Results: After initiating corrective measures in 2008, a progressive reduction in the percentage of non-conforming samples was observed from 2009 onwards. Despite a 127.84% increase in the total number of specimens received, there was a significant reduction in non-conforming samples from 0.29% in 2007 to 0.07% in 2015, resulting in an improvement of 75.86% [P <0.050]. In particular, specimen identification errors decreased by 0.056%, with a 96.55% improvement

Conclusion: Targeted educational activities directed primarily towards hospital nursing staff had a positive impact on the quality of laboratory specimens by significantly reducing pre-analytical errors

Portal vein thrombosis in adult Omani patients: a retrospective cohort study

Objectives: We sought to study the occurrence of portal vein thrombosis [PVT] in adult Omani patients

Methods: We conducted a retrospective cross-sectional study in patients diagnosed with PVT, which was confirmed by radiological imaging, from two tertiary hospitals over a 10-year period

Results: Amongst the 39 patients enrolled in the study, 15 [38.4%] had cirrhosis of the liver, and 24 [61.5%] were non-cirrhotic. In the non-cirrhotic PVT patients, 15 [62.5%] had acute PVT, whereas nine [37.5%] had chronic PVT. PVT was more common in males than females, [25 [64.1%] vs. 14 [35.8%], respectively, p = 0.020]. The three most common clinical symptoms were abdominal pain [n = 25, 64.1%] followed by nausea [n = 12, 30.7%] and fever [n = 8, 20.5%] patients. Causative risk factors included prothrombotic states [17.9-28.2%] and local factors [20.5%] such as cholecystitis, cholangitis, and liver abscess. Complications were found in 23.0% of patients with PVT, namely variceal bleeding in seven patients [17.9%] patients and bowel ischemia in two patients [5.1%]. Management with sclerotherapy was performed in all patients with variceal bleeding. Thrombectomy was done for one patient complicated with intestinal ischemia, but as it failed, he was treated with warfarin anticoagulation

Conclusions: This is the first study reflecting a real-life practice in PVT with possibly underlying inherited and acquired prothrombotic conditions as well as complications due to local and malignant conditions from Oman. We studied the prevalence, clinical presentation, underlying possible etiological factors, treatment, and outcomes. Since causative factors were found in 36 patients [92.3%], etiological screening seems worthwhile in every case with PVT, but thrombophilia screening may not be cost-effective

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

Haemostatic parameters in patients with behget's disease

This study aimed to evaluate the cause of thrombosis in Behcet's disease [BD] patients, since abnormalities in coagulation and fibrinolytic parameters have shown contradictory results. Haemostatic parameters were retrospectively evaluated in BD patients treated between January 2007 and January 2011 at Sultan Qaboos University Hospital, Oman. The blood samples of 35 Omani BD patients and 30 healthy controls were analysed for factor VIII:C levels, activated protein C resistance [APCR], von Willebrand factor [vWF] antigens [Ag], collagen binding and ristocetin co-factor activity [RiCoF], antithrombin [AT] protein C [chromogenic and clotting], protein S, homocysteine, tissue plasminogen activator, plasminogen activator inhibitor, plasminogen, alpha 2-antiplasmin, lupus anticoagulant and anticardiolipin and beta2-glycoprotein-l antibodies. The mean values of factor VIII:C, vWF Ag, AT and protein S were significantly higher in the patient group [P = 0.01, 0.006, 0.04 and 0.01, respectively]. There was no deficiency in protein C. Screening for APCR, anticardiolipin antibodies, anti-beta2-glycoprotein-l antibodies and lupus anticoagulant was negative and there were no differences in homocysteine levels, nor were there differences between patients with and without thrombosis. Six patients had elevated factor VIII:C levels [>150 lU/dL, P <0.02] which normalised on repeat measurements after three months. The elevation of factors VIII:C, vWF Ag and AT most likely represent an acute phase phenomenon. In this study, thrombophilic factors did not seem to explain thrombotic tendency. Therefore, further mechanistic studies in a larger group of patients are needed to elucidate the basis for thrombosis in BD. We hypothesise that active BD causes vasculitic endothelial perturbation with dysfunction, leading to the observed increased propensity for thrombosis

Transient myeloproliferative disorder and Down Syndrome. Is there a link?

An extremely premature male neonate presented with an unusual multisystem dysfunction within the first 24 to 48 hours of life. The unfolding of clinical events and investigations revealed a transient myeloproliferative disorder [TMD]. TMD was the main indication for karyotyping of this premature infant without clinical symptoms of Down syndrome. The awareness of TMD in a newborn warrants karyotype analysis to look for trisomy 21 and a close surveillance because of its potential progression to true leukaemia

Complications of PORT-A-CATH in patients with sickle cell disease

Red cell exchange/transfusion is frequently used in the management of patients with medical complications related to acute severe sickle cell disease [SCD]. However, peripheral venous access is often difficult without central venous catheters [CVCs] in adult patients with moderate or severe SCD. To review our experience with the use of the PORT-A-CATH device in sixteen patients with SCD undergoing exchange or simple transfusions. Among a cohort of 550 patients who frequently visited the inpatient service, sixteen SCD patients required the insertion of a PORT-A-CATH device. These patients included 3 males and 13 females, aged 25-44years [31.1 +/- 2.3; mean +/- [SD]. A total of 24 PORT-A-CATH devices were implanted in these 16 patients during the study period. Eleven patients had 1 device implanted, three patients had 2 devices, one patient had 3 devices, and one patient had 4 devices implanted. Out of the 24 devices implanted, 17 required removal, due to either infection associated with sepsis and/or thrombosis. The organisms involved were Candida spp. [3], C. Parapsilosis [2], C. albicans [1], C. famata [1], C. lusitanice [1], Staphylococcus spp. [6], and S. aureus [3], as well as the coagulase-negative Staphylococcus [2], alpha hemolytic Streptococcus [1], Diphtheroidbacilli [2], Pseudomonas aeruginosa [2], Ps. Spp. [3], Escherichia coli [3], Klebsiella oxytoca [1], Klebsiella pneumoniae [1], Klebsiella spp. [1], Serratia liquefaciens [1], Serra-tia fanticola [1], Achromobacter spp. [2] Chromobacterium violaceum [1], Delftia acidovirans [1], Stenotrophomonas maltophile [1], Alcaligenes faecalis [1], and Enterobacter cloacae [1]. Two episodes of documented thrombosis were observed

Complete recovery following sudden sensorineural hearing loss in a patient with sickle cell disease

A sickle cell disease [SCO] patient with vaso-occlusive crisis [VOC] developed sudden senserinural hearing loss [SNHL], which responded well to a rapid course of corticosteroids along with exchange transfusions. The otolaryngologist should be aware of the otologic manifestations of SCD such as SNHL A good response to steroids, which averted a permanent hearing loss, warrants further studies to define the augmentive benefits of such therapy along with exchange transfusions in patients with SCD in VOC who develop sudden SNHL

Neonatal screening: mean haemoglobin and red cell indices in cord blood from Omani neonates

The aim of this study was to validate the interpretation of red blood cell indices in complete blood count [CBC] and high performance liquid chromatography [HPLC] results on cord blood samples in consecutive Omani neonates. Cord blood samples from 7,837 neonates, were analysed with CBC and HPLC using the beta-thalassaemia short programme. Direct sequencing of abnormal samples with HbS, HbD, HbE and HbC was performed to validate the HPLC results. Additionally, in cases with HbA <10%, the beta-globin gene was directly sequenced for beta-thalassaemia mutation analysis. Overall, 4,042 subjects [51.58%] had normal HPLC [HbA 22.88 +/- 8.03; HbF 77.02 +/- 8.04], whereas the presence of Hb Barts in the remaining 3,795 cases [48.42%] indicated the presence of alpha-thalassaemia. No case of HbH was detected. In the former subgroup respectively, the mean Hb [15.38 +/- 2.04 g/dl] red blood cell [RBC] count [4.69 +/- 0.68 x 10[12]/l], Hct [50.5 +/- 7.18%], mean corpuscular volume [MCV] [107.66 +/- 7.75 fl], mean corpuscular haemoglobin [MCH] [33.31 +/- 4.07 pg], mean corpuscular haemoglobin concentration [MCHC] [30.98 +/- 3.44 g/dl], red cell distribution width [RDW] [17.01 +/- 2.17%] whereas, in the latter group with alpha -thalassaemia, it was [14.79 +/- 2.90 g/dl]; [5.09 +/- 0.77 x 10[12]/l]; [49.7 +/- 7.40%]; [97.29 +/- 13.8 fl]; [29.74 +/- 11.80 pg]; [30.39 +/- 3.6 g/dl], and [18.09 +/- 2.56%] respectively. DNA sequencing of samples with abnormal haemoglobin could validate the CBC and HLPC interpretations in all cases. This is the first study comparing the hemoglobin and red cell indices in the cord blood from newborn Omani subjects with those from other countries in the region, showing comparable results to those seen in Saudi neonates. The study also validates the CBC and HPLC interpretations of the cord blood red cell indices in the Omani neonate. The incidence of alpha-thalassaemia diagnosed by the presence of Hb Barts in cord blood of neonates was 48.42%