Sickle-cell disease (SCD) is a wide-spread inherited hemolytic anemia that is due to a point mutation, leading to the substitution of valine for glutamic acid, causing a spectrum of clinical manifestations in addition to hemolysis and anemia. Acute painful crisis is a common sequela that can cause significant morbidity and negatively impact the patient's quality of life. Remarkable improvements in the understanding of the pathogenesis of this clinical syndrome and the role of cell adhesion, inflammation, and coagulation in acute painful crisis have led to changes in the management of pain. Due to the endemic nature of SCD in various parts of the Middle East, a group of physicians and scientists from the United States and Middle East recently met to draw up a set of suggested guidelines for the management of acute painful crisis that are reflective of local and international experience. This review brings together a detailed etiology, the pathophysiology, and clinical presentation of SCD, including the differential diagnoses of pain associated with the disease, with evidence-based recommendations for pain management and the potential impact of low-molecular-weight heparin (LMWH), from the perspective of physicians and scientists with long-term experience in the management of a large number of patients with SCD.

VTE remains a common problem in medical and surgical patients in the Arabian Gulf states. Recognition of the common risk factors is of extreme importance to implement the appropriate prophylactic strategy according to the published guidelines.

Background: The Global Risk Profile Verification in Patients with Venous Thromboembolism (GRIP VTE) was the first prospective multicenter registry conducted in five Gulf countries to explore the epidemiology of venous thromboembolic (VTE) disorders and to provide data on diagnosis and disease management. Methods: Data on 242 patients with confirmed VTE were submitted between September 2003 and November 2003 by multidisciplinary specialists from 28 contributing hospitals in the Gulf region (Kuwait, Bahrain, Qatar, Oman, and the UAE). Patients with a suspected diagnosis of VTE were included. The data management team at a sponsor-independent study coordinating center ensured data quality. Differences between groups were assessed by the Chi square test or Fisher exact test for categorical variables. The Student t-test was used for testing proportions. A two-tailed P value <0.05 was considered significant. Doppler ultrasound and lung scans were the most preferred modalities in the diagnosis of deep vein thrombosis (DVT) and pulmonary embolism (PE). Results: The table shows the incidence of VTE. The most common symptoms of DVT and DVT/PE patients were calf pain (72%), calf swelling (63.8%), and localized tenderness (52.2%). Calf pain and localized tenderness were significantly greater (P <0.001) in cases of DVT alone than in cases of DVT/PE. The most common symptoms in patients with PE alone and DVT/PE were dyspnea (83.6%), thoracic pain (69.1%), and cough (40%). Cough and hemoptysis occurred more frequently in PE cases than in cases of DVT/PE (P <0.001). Risk factors for VTE were immobilization >3 days (41.3%), age >65 years (28.9%), a history of VTE (20.7%), and trauma (19%). Surgical intervention in the previous year was an independent risk factor for VTE, 83.8% of such patients experiencing VTE within 4 weeks of surgery. There was a strong association between VTE and orthopedic procedures (P=0.0016). Among surgical interventions, orthopedic procedures induced the greatest number of VTE cases, followed by general surgical procedures and gynecological procedures. Low molecular weight heparins (LMWHs) were chosen to treat 33.7% of DVT cases, while unfractionated heparin (UFH) was used in 21.9% of cases. UFH use in PE and DVT/PE was 57.1% and 55%, respectively, and LMWHs use was 14.3% each for PE and DVT/PE. Oral anticoagulant use in DVT/PE, DVT, and PE was 30%, 19.8%, and 2.9%, respectively. Conclusion: The main risk factors predisposing to VTE are immobilization, age >65 years, a history of VTE, and trauma. The highest incidence was observed in medical patients, necessitating prophylaxis in patients at risk. Previous surgical interventions were independent risk factors for VTE, requiring extended prophylaxis, including outpatient thromboprophylaxis, in patients undergoing extensive surgical procedures. Incidence of DVT, PE, and DVT/PE DVT PE DVT/PE Frequency of cases - n (%) 187 (77.27%) 35 (14.46%) 20 (8.26%) Departments Medical 74 (39.5%) 19 (54.28%) 10 (50%) Surgical 61 (32.62%) 9 (25.71%) 6 (30%) Others 52 (27.8%) 7 (20%) 4 (20%)

Acquired thrombotic thrombocytopenic purpura (aTTP) is a rare hematological emergency that is characterized by microangiopathic hemolytic anemia (MHA), thrombocytopenia, fever, and multiorgan failure due to autoimmune-mediated deficiency in ADAMTS-13 activity. Currently plasma exchange, with or without steroids, is the frontline option of management of aTTP that should be started promptly once the disorder is clinically-suspected. Besides, immunomodulators were studied in patients with aTTP to achieve stable remission and reduce the risk of relapse in patients with suboptimal response to plasma exchange; however, clinical trials showed equivocal results. in addition, published data on early diagnosis, referral, and treatment patterns of aTTP patients in Gulf Council Countries (GCC) are still lacking. Therefore, the present consensus report aimed to present an overview of aTTP situation in GCC by bringing together a panel of experts from three GCC to share their views on current trends and practice regarding aTTP. The experts discussed challenges including the lack of reliable data regarding the incidence of aTTP in GCC and delayed results of ADAMTS-13 activity testing. Limited patients’ access to tertiary centers and low level of awareness about the aTTP clinical spectrum among general practitioners are other challenges. The experts agreed that there is a need for national and regional consensus regarding the diagnosis and treatment of aTTP in Gulf region.