Sickle cell disease (SCD) is a genetic disease influenced by ethnicity and regional differences in its clinical course. Recent advances in the management of SCD with newer therapies are being introduced to the Western population. However, many of these treatments are yet to be used in the Arabic SCD population. Understanding the genetic variations of SCD regionally is essential to anticipate the utilization of new treatments. This systematic review’s main objective is to pool the available data on the genetic composition of SCD in the Arabic population. Data for 44,034 patients was extracted from 184 studies (11 case reports, 8 case series, 56 retrospectives, 107 prospective observational studies, and 2 clinical trials) using PubMed, Scopus, and Google Scholar. Male (49%) and female (51%) patients were equally reported wherever gender was available (N=13105). Various SCD genotypes were reported in a total of 14,257 patients, including Hb SS (77%) Hb Sβ 0 (9.9%), and Hb Sβ+ (7.2%), while the rest of the genotypes, including HbSC, HbSD, HbSE, HbSO Arab, Hb S/α-Thal, Hb Sβ 0 + α-Thal, and HBS Oman were individually reported in < 4% of the cases. Major SCD complications in the Arab population included pain crises (48.25%) followed by neurological complications (33.46%), hepatobiliary complications (25.53%), musculoskeletal complications (24.73%), and hemolytic anemia (23.57%). The treatments reported for SCD included hydroxyurea (20%), blood transfusion (14.32%), and Deferasirox (3.03%). We did not find the use of stem cell transplantation or newer treatments such as L-Glutamine, Voxelotor, Crizanlizumab, or gene therapy reported in any of the studies included in our review. This review highlights the genetic makeup of SCD in Arab countries and its common phenotypic manifestations and will help direct further research on SCD in this region, especially concerning genetic therapy. Systematic Review Registration: The protocol has been registered in the International Prospective Register of Systematic Reviews(PROSPERO):CRD42020218,666. https://www.crd.york.ac.uk/PROSPERO/display_record.php?RecordID=218666.

Avascular necrosis (AVN) is a debilitating complication in sickle cell disease (SCD) patients, and its management is usually challenging. This study aims to evaluate the clinical and epidemiological features and therapeutic options of AVN in sickle cell patients in Qatar. A cross-sectional study was conducted on a 49 SCD patients who were diagnosed with AVN and attended the hematology clinic at the National Center for Cancer Care & Research, Hamad Medical Corporation, Qatar between Jan-2011 to Jan2021. Forty-nine adult patients with SCD who were diagnosed with AVN were studied. The median age of the study population is 32 years, and the median age at the first AVN diagnosis was 26 years (range: 11-44 yr.). 37 (75.5%) patients suffered from multiple joints AVN while 12 (24.5%) had single joint involvement. 31 (63.3%) patients had bilateral hip AVN and 18 (36.7%) had shoulder involvement. 30 patients (61%) were on hydroxyurea treatment. Based on Ficat and Arlet classification of AVN, 57 % of patients had stage III and above at first diagnosis. 20 (40.8%) were managed with a conservative approach, 11 (22.4%) received hyperbaric oxygen with good response, 6 (12.2%) underwent hip core decompression and 12 (24.5%) underwent hip replacement surgery. In SCD patients, AVN occurred more during the 3^rd and 4^th decades of life. The majority of AVN represented with advanced stage and had multiple joint involvements. We recommend adopting a low threshold of joint imaging for early detection and prevention of further complications. (www.actabiomedica.it)

Tumor lysis syndrome (TLS) is a hematological emergency. This syndrome is characterized by metabolic derangements such as hyperkalemia and hypocalcemia, which result from rapid lysis of cells, especially rapidly growing tumors, after the initiation of chemotherapy. It is rarely seen in chronic myeloid leukemia (CML) and has not been previously reported to be triggered by coronavirus disease 2019 (COVID-19) infection. We report a case of a 45-year-old male, a known case of CML in the chronic phase, who presented with fatigue and abdominal pain for four days. Initial laboratory results were consistent with leukocytosis and positive COVID-19 antigen. The patient was started on intravenous fluids and hydroxyurea; however, over the next few days, he deteriorated quickly and developed oliguric acute kidney injury (AKI) with electrolyte disturbance consistent with TLS. The patient was shifted to the intensive care unit and underwent one sustained low-efficiency dialysis (SLED) session and received rasburicase. Over the next few days, the patient started to improve and was discharged in good shape. Although CML rarely presents with TLS, physicians should monitor their patients closely, especially those who have concurrent COVID-19 infection, as this condition may result in lethal sequelae such as AKI, severe arrhythmias, and multiorgan failure. Additionally, early detection and treatment lead to a better prognosis.

Autoimmune hepatitis (AIH) is a chronic disease characterized by hepatocellular inflammation and destruction. Its pathogenesis remains unclear. Current evidence suggests that environmental factors, including viral infections, are implicated as possible triggers. We present a case of a 36-year-old lady with systemic lupus erythematosus who presented with abdominal pain, nausea, vomiting, and jaundice. She was initially diagnosed as acute hepatitis E virus (HEV) infection based on positive serology, but she did not improve with conservative management. Subsequently, she underwent liver biopsy that showed features characteristic of AIH. We discuss the role of HEV as a possible trigger of AIH. We also highlight the possibility of cross-reactivity between liver antigens and HEV as an explanation for the positive HEV serology in AIH. We explore the management options of cases in which both acute HEV infection and AIH coexist, which might be challenging as there is no current consensus whether to start immunosuppressive therapy or to manage such cases conservatively. Moreover, we discuss previous similar cases in which different lines of management were chosen.

Immune thrombocytopenic purpura (ITP) is a blood disorder in which antibodies coating platelets cause platelets destruction in the spleen with resultant low platelets count and an increased tendency for bleeding. Coronavirus disease 2019 (COVID-19) is an illness caused by SARS-COV2; it was first identified in December/2019; though it mainly affects the respiratory system, multisystemic complications are identified. Several ITP cases post mRNA SARS-CoV-2 vaccines were reported, and different pathophysiology theories about the underlying pathophysiology were discussed, but only a few ITP relapse cases have been reported so far. We present a 28-year-old Asian male, a known patient of ITP and in partial remission for eighteen months, who presented to the emergency department with ITP relapse (platelets count of 1 × 10^3 /µL), four days after receiving the second dose of Pfizer SARS-CoV-2 vaccine, which required treatment with intravenous immunoglobulins and dexamethasone. We further discuss the preferred approach in ITP patients who are willing to receive the COVID-19 vaccine.

Coronavirus disease 2019 (COVID-19) is caused by a virus known as severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Since the first pandemic wave, SARS-CoV-2 had developed significant changes and mutations that resulted in the emergence of different strains. Each strain varies in its virulence and disease severity. Most reports have shown that the Omicron variant causes mild illness. Little is known about the impact of Omicron in patients with chronic myeloid leukemia. We present patients with chronic myeloid leukemia who had infection with the Omicron variant of the SARS-CoV-2 and their outcomes. Retrospective data from the records of the National Center for Cancer Care and Research from December 20, 2021, to January 30, 2022. Participants were adults over the age of 18 years with Omicron infection who had been diagnosed with chronic myeloid leukemia according to World Health Organization classifications from 2008 and 2016. Eleven patients with chronic myeloid leukemia had Omicron infection. All patients had a mild disease according to the World Health Organization classification of COVID-19 severity. The majority of patients were young males. In patients with chronic myeloid leukemia, infection with the Omicron variant of the SARS-CoV-2 usually results in mild disease not requiring hospitalization.