OBJECTIVE: Interleukin-10 (IL-10) gene polymorphisms might play a part in the development of some malignant tumors. It has been linked with high bcl-2 expression in some B-lymphocyte malignancies. Its relationship with chronic lymphocytic leukemia (CLL) development is still under investigation. Other studies have linked Splicing Factor 3B Subunit 1 (SF3B1) mutations to a poorer prognosis of CLL. From this context, we have great interest to investigate the effect of both IL-10 (1082G/A) and SF3B1 (2098A/G) gene polymorphisms on CLL in this study. MATERIALS AND METHODS: Peripheral blood mononuclear cells were analyzed for IL-10 (1082G/A) and SF3B1 (2098A/G) gene polymorphisms by real-time quantitative polymerase chain reaction in 80 newly diagnosed CLL patients and 80 controls. RESULTS: Our results showed that the IL-10 (G/A) genotype, IL-10 (A/A) genotype and IL-10 A allele and SF3B1 (A/G) genotype and SF3B1 G allele were increased significantly in the patients group compared with the control group. CONCLUSION: IL-10 gene polymorphisms (1082 G/A and A/A) and A alleles might be associated with increased risk of CLL development compared with G/G genotypes and G alleles and are a probable risk factor for the disease. Also, our study demonstrated that SF3B1 (2098A/G) polymorphisms and G allele are related to and might be a causative factor for CLL.

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INTRODUCTION: The success of tyrosine kinase inhibitors in the treatment of chronic myeloid leukemia (CML) has increased the focus on survivorship and late toxicity in oncological care. Cardiovascular (CV) health has emerged as an important consideration in patients of CML. Some studies have shown an increased incidence of CHF with Imatinib therapy. This study was conducted to assess the cardiac functions in patients of CML on Imatinib therapy. METHODS: Seventy newly diagnosed CML patients started on Imatinib therapy having normal baseline cardiac functions as assessed by two-dimensional echocardiography and multigated acquisition (MUGA) scan were included in the study. Further assessment of cardiac functions at 3, 6, and 12 months was done by MUGA scan. RESULTS: At 3, 6, and 12 months of follow-up, 7.14%, 10%, and 11.43% of patients, respectively, showed evidence of cardiotoxicity in the form of reduced ejection fraction and wall abnormalities on MUGA scan (P = 0.58, 0.013, and 0.006, respectively). CONCLUSION: Cardiac dysfunction was seen in 11.43% of patients on Imatinib therapy for CML, though none of them were symptomatic. CML patients on Imatinib therapy must be monitored for cardiac functions, especially if they have other CV risk factors.

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INTRODUCTION: Sickle cell disease (SCD) is an autosomal recessive disorder characterized by hemolytic anemia with abnormal red blood cells. Stroke is a devastating complication of SCD. In Saudi Arabia, there is a high incidence of children with SCD who have had stroke. No studies have been conducted to look at the risk factors. In this review, we aim to explore these risk factors. METHODOLOGY: In this retrospective cohort, individuals between 0 and 18 years old, diagnosed with SCD and had either a stroke, or pathological transcranial Doppler ultrasonography (TCD) result (cerebral blood velocity ≥200 cm/second or repeated borderline result >170 cm/second) were included. 38 cases were assessed. The parents were interviewed regarding patients personal, social, and family history. Medical and laboratory data were obtained from medical records. RESULTS: Females were more affected (20/38). Most children are of an average socioeconomic status (20/38 compared to only 12/38 with poor socioeconomic status). Parental education inversely correlated with the risk of stroke/pathological TCD. The most common presentation of stroke was weakness (9/38), followed by headache (5/38). History of a prior stroke was found to be significant at 15.8%. The Cooperative Study of SCD found that leukocytosis is associated with an increased risk of stroke. In contrary, our review showed an average white blood cell count of 13.01 (+/‒5.36), HbS concentration at the onset of stroke, and/or pathological TCD of 66.23% (+/‒20.11). The most common artery involved was the middle cerebral artery. CONCLUSION: Stroke is very prevalent in children with SCD in Saudi Arabia. A striking finding was that parental education inversely correlated with the risk; therefore, improving the awareness level of SCD among parents is crucial. Wide implementation of TCD as a screening tool and improving compliance with TCD may lead to reduce the risk. We encourage Saudi researchers to focus on central nervous system complications in children with SCD.

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BACKGROUND: Psoriasis has a multifactorial pathogenesis encompassing genetic, environmental, and immunological factors. There is a dire need for specific, cost effective, reliable, and universally accepted laboratory marker as indicator of severity of psoriasis. MATERIALS AND METHODS: A cross-sectional study was conducted on 50 psoriasis patients and 50 healthy controls. Hematological parameters including platelet indices (platelet count [PC], plateletcrit, mean platelet volume [MPV], platelet large cell ratio, platelet distribution width [PDW]), neutrophil–lymphocyte ratio (NLR), platelet–lymphocyte ratio (PLR), and red blood cell distribution width (RDW) were evaluated and correlation of these indices among themselves and with Psoriasis Area and Severity Index (PASI) analyzed. Statistical analyses were performed using SPSS version 19.0 (SPSS Inc., Chicago, IL, USA). RESULTS: A statistically significant difference in RDW, PC, MPV, PDW, NLR, and PLR was observed between psoriasis versus controls and mild versus moderate to severe psoriasis. There was a significant positive correlation between PASI and RDW, MPV, platelets, PLR while erythrocyte sedimentation rate showed a significant correlation with MPV. MPV and RDW, RDW and NLR, and RDW and PLR were also found to be correlated. MPV showed highest sensitivity and specificity both. MPV (area under the curve: 0.970, P < 0.001) demonstrated better predictive power as per area under curve of receiver-operator curve as compared to other parameters for psoriasis. CONCLUSION: The present study assessed the role of simple and low-cost parameters easily computed from routine tests like complete blood count as biomarkers for severity of psoriasis. Mean values of MPV, RDW, NLR, and PLR were found to be higher in psoriasis patients compared to controls. Moreover, a significant correlation was observed between PASI and these novel markers. However, additional large-scale, multicenter studies need to be conducted before application of these parameters in clinical practice.

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BACKGROUND: Immune tolerance induction (ITI) is the gold standard approach for eradicating inhibitors and increasing patient tolerance to factor VIII. The success rate of ITI may vary depending on patient variables and factors relating to the pattern of treatment for the induction of immune tolerance. Children with recently diagnosed inhibitors are the best candidates for ITI, and those with favourable expected results should be offered ITI as soon as inhibitors are identified. Recombinant factor VIII Fc fusion protein has proved a therapeutic advantage in patients with high factor VIII inhibitor titers AIMS AND OBJECTIVES: To evaluate the clinical characteristics and outcomes of ten hemophilic pediatric patients who underwent ITI therapies to eliminate FVIII inhibitors at Tawam Hospital, UAE MATERIALS AND METHODS: The data of ten hemophilia A children aged 2–7 years with high inhibitor titers who underwent ITI therapy at Tawam Hospital, UAE, were retrospectively collected for this case series. A comparison of bleeds before and after the ITI therapy was also made. Patients with either failed or partially successful primary ITI therapy underwent rescue ITI therapy. Data analysis was performed using SPSS version 26. RESULTS: Full success was achieved in 60% (6/10) of the patients, 10% (1/10) achieved partial success, whereas 30% (3/10) failed the primary ITI therapy. The rescue ITI therapy was successful in 50% (2/4) of the patients and the remaining 50% (2/4) achieved partial success [Table 2]. The rescue ITI was successful in 66% (2/3) of those patients who received Elocta and partially successful in 33% (1/3) CONCLUSION: ITI therapy is the gold standard for the eradication of antibodies against FVIII. The patients with good expected outcomes should be offered ITI as soon as the inhibitors are confirmed. The use of extended half life rFVIIIFc demonstrated therapeutic benefit, particularly in challenging ITI patients with high inhibitor titers

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Anemia is a common finding in our population. Females are highly predisposed to develop anemia. Evaluation of anemia due to a single cause may be straightforward in most of the situations, however rare causes cannot be ruled out. In this case report, we present to you a case of a 35-year-old female who came with complaints of breathlessness. On further evaluation, she had pallor, icterus, and mild splenomegaly. Her hemoglobin (Hb) was later found to be 8.1 g/dl, pyruvate kinase activity was normal, normal liver enzymes, Heinz body positive, and elevated retics count. Thus, she was found to have a rare form of anemia, i.e., Heinz body hemolytic anemia. She was advised for splenectomy and started on medications. Following the procedure, her Hb started to rise to 9.6 g/dl and reached to 13.8 in the following months.

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