Bleeding disorders are infrequent health problem in most of the societies and gain less focus by medical professionals. There are some data suggesting a higher prevalence of mild bleeding symptoms and coagulation factors deficiency in societies with high consanguinity. Furthermore, few validated tools for detecting bleeding disorders through large sample sized surveys are available in Arabic language. Hereby, we are presenting our experience of translating and validating one of these tools for implementation into an Arabic speaking population. An expert committee was formulated with a primary objective of smoothly translating the questionnaire into Arabic while maintaining the reliability and validity of the instrument. Pearson's correlation coefficient was calculated to test for the significance of correlation found in test-retest reliability. In test-retest reliability assessment, we did not find the individuals' responses changing over time. Content validity was confirmed through a panel of practicing hematologists. Establishment of an expert committee to oversee the process of translation and adaptation of a foreign language questionnaire into Arabic has proven its usefulness.

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The hallmark of diagnosing classical chronic myelogenous leukemia (CML) is the identification of Philadelphia (Ph) chromosome (Chr). However, some CML cases show additional structural/numerical chromosomal abnormalities involving either Ph Chr or more frequently other Chrs. Genetic alterations sparing Ph Chr are commonly called “additional chromosomal abnormalities (ACAs)” which have been extensively analyzed in recent large studies. In contrast, the presence of additional genetic abnormalities in Ph Chr has been anecdotally reported with an ambiguity of their impact on treatment response. In our case, we report a newly diagnosed CML patient with the rarest additional chromosomal aberration affecting Ph Chr which has revealed an optimal response over a period of 2 years of follow-up. This report underlines the importance of re-examining CML cases for any ACAs, especially those occurring in Ph Chr, which might be overlooked easily. Moreover, their possible role in disease prognostication should be sought, as well.

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BACKGROUND: In India, hemoglobinopathy screening programs focus on reducing the burden of beta-thalassemia by screening the antenatal females and young adolescents. Although alpha (α)-thalassemia is common in the Mediterranean countries, alpha (α)-thalassemia screening and diagnosis are performed in a few centers in India. HbH disease is an uncommon clinical phenotype of α-thalassemia with variable clinical presentation. The hallmark feature of HbH is the presence of golf ball inclusions in the red cells demonstrated with supravital staining. PATIENTS AND METHODS: Here, we discuss an algorithmic approach to screen hemograms for thalassemic indices, followed by evaluation of reticulocyte preparation after 40 minutes (supravital stain preparation for golf ball inclusions). If golf ball inclusions were positive, Hb analysis on Hb-capillary zone electrophoresis (Hb-CZE)was done. RESULTS: We received 28,000 samples for complete hemogram and blood film examination, over a period of 8 months. Reticulocyte count was required in 11,600 samples out of which 13 cases were positive for golf ball inclusions. Of these, nine were identified as HbH disease on Hb-CZE. These patients presented with pallor and generalized weakness. On examination, jaundice (3/9 patients) and hepatosplenomegaly (4/9 patients) were relatively uncommon. The majority of them (6/9) required infrequent blood transfusions. All patients had microcytic hypochromic red cell indices with median HbA of 89% (range: 68.1%–96.9%), HbA2 of 1.4% (range: 0.8%–1.7%), and fast-moving band in zone 15 (HbH) of 5.6% (range: 1.4%–30.3%) on Hb-CZE. CONCLUSION: We emphasize a systematic screening of hemograms and peripheral smears along with a simple and cost-effective screening test for golf ball inclusions to diagnose HbH disease that often poses a clinical dilemma due to its variable clinical presentation.

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CONTEXT: Worldwide, chronic myeloid leukemia (CML) accounts for 15% of all leukemia types. CML treatment and outcome has improved dramatically from the average survival of 3–5 years in the past to almost normal life expectancy with the new targeted therapy with tyrosine kinase inhibitors (TKI). Compliance of CML patients with oral TKIs is a medically challenging issue preventing achieving a favorable outcome. AIMS: The aim of this study is to assess CML patients' adherence to treatment in King Abdulaziz Medical City (KAMC). SUBJECTS AND METHODS: This is a prospective, observational study that includes all patients diagnosed with CML in KAMC-Riyadh (KAMC-R) where patients were interviewed before receiving TKI prescription and during a subsequent visit. Consent, demographics, and medication data were obtained in the first encounter. Compliance and reasons for noncompliance were assessed using pill count methods during the second visit. STATISTICAL ANALYSIS USED: Statistical analysis was performed using SPSS. RESULTS: A total of 63 CML patients are followed and treated in KAMC-R at the time of conducting this study. Sixteen patients were excluded from the study due to either loss of follow-up or using a non-TKI treatment option. The study was conducted on 47 patients; male:female ratio was 20 (40%):28 (60%). The mean age was 51 years (range 23–76 years). Twenty-four patients (51%) were fully compliant to the TKIs while 23 patients (49%) were not compliant. The study reported three main reasons for noncompliance, drug toxicity in 7 patients (30%), forgetfulness in 7 patients (30%), and ignorance in 2 patients (9%). CONCLUSIONS: To the best of our knowledge, this is the first study assessing compliance of CML patients with TKIs among Saudi population. Noncompliance with TKIs is a major health-care problem affecting the outcome of treatment. Stressing on patient education before and during TKIs treatment might improve the compliance.

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In response to the high prevalence of atherothrombotic diseases and the residual risks left by the current antiplatelet therapies, the Food and Drug Administration approved the use of vorapaxar as a secondary prevention of cardiovascular events. Vorapaxar is a novel oral antiplatelet agent that antagonizes protease-activated receptor-1 and inhibits platelet activation without influencing coagulation parameters. In this review, we summarized findings of the main studies and the subanalyses from two pivotal phase III clinical trials of vorapaxar, as well as the results of the available observational studies to assist treatment decision-making of vorapaxar for clinicians. Evidence shows that vorapaxar treatment brings favorable results to patients with myocardial infraction history, peripheral arterial diseases, and acute coronary syndrome and those who underwent coronary artery bypass grafting surgery. Studies supported the efficacy of vorapaxar in treating incident coronary stent thrombosis, acute limb ischemia, and peripheral revascularization which outweighed the safety concern of bleeding. However, vorapaxar should not be administered to patients with prior stroke or transient ischemic attack due to excessive risks of bleeding. Future studies should focus on examining the long-term risks and benefits of vorapaxar on single cardiovascular outcomes and the optimal dosage of vorapaxar for different patient populations.

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Hemophagocytic lymphohistiocytosis (HLH) is a rare, life threatening condition characterized by immune dysregulation and benign proliferation of phagocytic cells, macrophages, and histiocytes. Lymphocytes (CD8 T-cells and natural killer cells) also play a major role in HLH. The ensuing cytokine storm and blood cells phagocytosis can lead to fatal multiple organ failure. So any diseases that can lead to activation of these cytokines can lead to hemophagocytic syndrome. 5 adult patients presented to Dr. Soliman Fakeeh Hospital, Jeddah, Saudi Arabia during 2014–2016 and fulfill the diagnostic criteria of HLH according to HLH-2004 diagnostic and therapeutic guidelines for HLH , have been included in this case series. Two out of five had viral infection, one patient was diagnosed with tuberculosis, and one patient developed secondary HLH in due to malignancy. The fifth patient did not have an identiable etiology. All of our patients presented with different symptoms and were diagnosed based on the standard approved criteria. Only two Out of five patients survived and remained disease free for a follow-up period of 24 months. As a conclusion, HLH syndrome is a rare condition with a high mortality rate. Aggressive treatment approach and early Intensive Care Unit admission is strongly recommended in patients with the cardinal diagnostic features of this condition.

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