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

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

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

Umbilical cord blood banking and transplantation: a short review

It is more than 20 years since the first cord blood transplant [CBT] was performed, following the realisation that cord blood [CB], which is normally wasted, is rich in progenitor cells and capable of rescuing haematopoiesis. Since then it has been appreciated that CB is rich in stem cells, and has many other features not the least of which is its ability to rescue the transplanted patient without a rigid need for full human lymphocyte antigen [HLA] compatibility. Also it is easily accessible, relatively free from infections and poses no medical risk to the donor. However, the quantity of the stem cells is rather small, thus predominantly restricting its use to children or adults requiring double units. In Oman, we have taken a keen interest in stem cell research and also CBT. We see such activities as an avenue for our patients, for whom a compatible bone marrow [BM] or a peripheral blood donor cannot be found, to have an alternative in the form of CBT. This has encouraged us to establish a national voluntary cord blood bank [CBB] which is a valuable option open to a selected group of patients, as compared to the controversial private CBB. This national CBB will have a better representation of HLA-types common in the region, an improvement on relying on banks in other countries. Considering the need for stem cell transplant/therapy in this country, it is only appropriate that this sort of bank is established to cater for some of these requirements

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%

Successful use of alternative anticoagulants in the management of heparin-induced thrombocytopenia with thrombotic complications report of 5 cases and review of literature

Heparin is one of the most frequently used anticoagulants. It is easy to use, but can be associated with life-threatening side effects. One of these is heparin-induced thrombocytopenia syndrome [HITS], which develops in about 3-5% of patients exposed to heparin and is associated with thrombosis in 1% of cases. We report here the successful treatment of five patients with HITS who were treated with alternative anticoagulants namely danaparoid or hirudin. The median time between their exposure to heparin and onset of symptoms and or signs was 10.2 days [range 7-14 days]. Platelet counts decreased to a mean of 38.4 x 10[9] /I [12-82 x 10[9]/1]. All five patients had evidence of thrombosis; four patients had clinical and radiological evidence of pulmonary emboli, one patient had confirmed deep vein thrombosis [DVT] and one patient had extensive skin necrosis of the thighs and abdomen. Platelet aggregation test were positive in two patients, inconclusive in one patient and negative in two patients. Two patients were anticoagulated with danaparoid and three with hirudin until their platelet counts returned to normal between 4 and 14 days [average 6 days] following the recognition of the syndrome. Our patients had significant morbidity, but no mortality. Immediate withdrawal of heparin is of paramount importance and introduction of alternative anticoagulant is necessary in the presence of thrombosis

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