A practical guide to the management of immune thrombocytopenia co-existing with acute coronary syndrome.

Introduction: Immune thrombocytopenia (ITP) management with co-existing acute coronary syndrome (ACS) remains challenging as it requires a clinically relevant balance between the risk and outcomes of thrombosis and the risk of bleeding. However, the literature evaluating the treatment approaches in this high-risk population is scarce. Methods and Results: In this review, we aimed to summarize the available literature on the safety of ITP first- and second-line therapies to provide a practical guide on the management of ITP co-existing with ACS. We recommend holding antithrombotic therapy, including antiplatelet agents and anticoagulation, in severe thrombocytopenia with a platelet count < 30 × 109/L and using a single antiplatelet agent when the platelet count falls between 30 and 50 × 109/L. We provide a stepwise approach according to platelet count and response to initial therapy, starting with corticosteroids, with or without intravenous immunoglobulin (IVIG) with a dose limit of 35 g, followed by thrombopoietin receptor agonists (TPO-RAs) to a target platelet count of 200 × 109/L and then rituximab. Conclusion: Our review may serve as a practical guide for clinicians in the management of ITP co-existing with ACS.

Pediatric Philadelphia-Negative Myeloproliferative Neoplasms in the Era of WHO Classification: A Systematic Review.

BACKGROUND: Philadelphia-negative myeloproliferative neoplasms (MPN) are most prevalent in the older population (median age at the diagnosis is above 60 years) and rarely diagnosed in pediatrics. Thus, our knowledge about the clinical presentation, mutational status, and complications of MPNs in pediatrics is limited. METHODS: The literature in English (PubMed, SCOPUS, and Google Scholar) was searched for studies, reviews, case series, and case reports of patients with Philadelphia-negative MPNs (including essential thrombocythemia, polycythemia vera, primary myelofibrosis, and profibrotic myelofibrosis) in the pediatrics age group (less than 18 years). Only studies that fulfilled WHO 2008 or 2016 criteria for MPNs were included. We aimed to describe the clinical characteristics, vascular and long-term complications, types of driver mutations, and treatment approaches in pediatric patients with MPNs. RESULTS: We reviewed 33 articles of available published literature from 2008 to 2022 and collected data from a total of 196 patients of the pediatric population. Among the cohort of patients, 139 had essential thrombocythemia (ET), 20 had polycythemia vera (PV), and 37 had primary myelofibrosis (PMF). The median age at the time of diagnosis for each disease varied, with 8.8 years for ET, 10 years for PV, and 3.6 years for MF. There was a slight difference in gender prevalence between both gender groups and all three diseases. The presenting symptoms were not mentioned in more than 50% of studies. We found that JAK2 was the most prevalent among all mutations. Both bleeding and thrombosis were present equally in ET, with 9% of cases complicated by bleeding and 9% complicated by thrombosis. Hemorrhagic events did not occur in patients with PV; thrombosis in children with MF was also not found. The progression into AML occurred in two patients with PV and one with ET. CONCLUSION: Given the rarity of MPNs in pediatrics and their different characteristics compared with adults, we believe there is a need for unique diagnostic criteria to match the different molecular statuses in pediatrics. Based on our review, the incidence of MPN complications in pediatrics, including thrombotic events, hemorrhage, and leukemic transformation, differs from that in adults.

The Impact of Tyrosine Kinase Inhibitors on Fatherhood in Patients With Chronic Myeloid Leukemia: A Mixed-Method Study.

INTRODUCTION: Multiple studies have demonstrated that tyrosine kinase inhibitors (TKIs) exert a significant extent of control over chronic myeloid leukemia (CML), as evidenced by studies such as the population-based Swedish CML registry, which found that patients reaching age 70 had a relative survival rate close to one when compared to the general population. Consequently, new perspectives on the safety of treatments have emerged, particularly in the context of their impact on fatherhood in men. According to the authors, this is the first study to examine the effect of TKIs on fatherhood in CML patients.  Methods: A single-center, mixed-design study (retrospective data review and phone interviews) was conducted with CML male patients in the chronic or accelerated phase, evaluating the effect of imatinib, dasatinib, and nilotinib on their fatherhood, irrespective of whether they were administered as a first, second, or third line of treatment. RESULTS: The study included interviews with 150 patients. Included were 27 patients. The average age was approximately 44.5 years. One hundred percent of the patients were in the chronic phase. The median age at first conception following TKI therapy was 36, and the median duration of TKI therapy was approximately seven years. The total number of offspring was 49; 98% were born at term and had a normal birth weight. No reports of stillbirths, fetal deaths, or congenital malformations were made. All the offspring grew and developed normally. No CML-related cancers were reported in any of the newborns. CONCLUSION: Around 98% of male CML patients receiving imatinib, dasatinib, or nilotinib did not experience a negative impact on their fatherhood or the health of their children. However, improved education for patients beginning treatment with TKIs addresses the potential psychological worry of having an unfavorable effect on their fertility or offspring, which may increase medication adherence.

Concurrent coronary artery disease and immune thrombocytopenia: a systematic review.

Introduction: Coronary artery disease (CAD) management in the setting of immune thrombocytopenia (ITP) remains very challenging to clinicians as a reasonable balance between bleeding and thrombosis risks needs to be achieved, and the evidence guiding such management is scarce. Methods: We conducted a systematic review following the PRISMA guidelines to summarize the available literature on the management and outcomes of CAD coexisting with ITP. We searched PubMed and Embase for studies published in English exploring CAD and ITP management until 05 October 2022. Two independent reviewers screened and assessed the articles for inclusion. Patients' characteristics, CAD treatment modalities, ITP treatment, and complications were reported. Results: We identified 32 CAD cases, among which 18 cases were revascularized with percutaneous coronary intervention (PCI), 12 cases underwent coronary artery bypass graft surgery (CABG), and two cases were managed conservatively. More than 50% were men, with a mean age of 61 ± 13 years and a mean baseline platelet count of 52 ± 59 × 109/L. Irrespective of the revascularization modality, most patients were treated with either corticosteroids alone, intravenous immunoglobulins (IVIG) alone, or in combination. Among those who underwent PCI, two patients had bleeding events, and one patient died. Similarly, among those with CABG, one patient developed bleeding, and one patient died. Conclusion: We found that revascularization with either PCI or CABG with the concurrent use of corticosteroids and/or IVIG for ITP was feasible, with an existing non-negligible risk of bleeding and mortality.

Clinical image report of acute Cholangitis in Beta-thalassemia major.

Beta-thalassemia is congenital red blood cell disorder. Gallstones is a recognized complication due to recurrent hemolysis. Acute cholangitis is a rare complication might occur in patient with beta-thalassemia. We report a case of acute cholangitis in patient with beta-thalassemia with Gilbert syndrome. We present a case of a young female of Arabic descent with acute abdomen. Workup revealed acute cholangitis with gallstones in the common bile duct. The ERCP was used to extract the stones than cholecystectomy.

Immune thrombocytopenia purpura flare post COVID-19 vaccine.

INTRODUCTION AND IMPORTANCE: Coronavirus disease 2019 (COVID-19) is a recently discovered disease that has yet to be thoroughly described. It is caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), a novel virus that can be transmitted easily from human to human, mainly by the respiratory route. The disease often presents with non-specific symptoms such as fever, headache, and fatigue, accompanied by respiratory symptoms (e.g., cough and dyspnea) and other systemic involvement. Currently, vaccination is the primary strategy to prevent transmission and reduce disease severity. However, vaccines have side effects, and the consequences of vaccination in different diseases are not well established. Moreover, the impact of SARS-CoV-2 vaccination during pregnancy is another not well-known area. CASE PRESENTATION: We present a young lady known to have ITP, which was controlled for years, presented with relapse after taking the SARS-CoV-2 vaccine during pregnancy. CLINICAL DISCUSSION: The patient had a relapse of ITP after the introduction of the first dose of the COVID-19 vaccine, which worsened further after the second dose. This suggests that patients with ITP who develop flare post-SARS-CoV-2 vaccine should have their second dose delayed, particularly if pregnant. CONCLUSION: To avoid further deterioration in platelet count, and avoid confusion due to the presence of different causes of thrombocytopenia and avoid complications related to thrombocytopenia during pregnancy which can affect the mode of delivery. THE CASE IS REPORTED IN LINE WITH THE SCARE 2020 CRITERIA: Agha RA, Franchi T, Sohrabi C, Mathew G, for the SCARE Group. The SCARE 2020 Guideline: Updating Consensus Surgical CAse REport (SCARE) Guidelines, International Journal of Surgery 2020; 84:226-230.

SARS-CoV-2 Omicron Variant in Patients With Chronic Lymphocytic Leukemia: Case Series.

The Coronavirus disease 2019 (COVID-19) is considered the largest pandemic in modern history. Since the first case was reported in 2019, several mutations have affected the severe acute respiratory syndrome-Coronavirus-2 (SARS-CoV-2), resulting in the emergence of new strains. These strains vary significantly in severity and transmissibility. The Omicron (B.1.1.529) variant was reported to cause mild disease in those affected, but little is known about the effect of Omicron on patients with chronic lymphocytic leukemia (CLL). We are reporting a case series of three patients with CLL who experienced infection with the SARS-CoV-2 Omicron variant and their outcomes.

A Case Series of SARS-CoV-2 Omicron Variant in Patients With Acute Leukemia.

Coronavirus disease 2019 (COVID-19) is a respiratory viral illness caused by coronavirus 2 (SARS-CoV-2). The disease often presents with non-specific symptoms, including fever, and fatigue, usually associated with respiratory symptoms (eg., cough) and other systemic involvement. The primary strategy to prevent transmission and reduce the disease severity of the SARS-CoV-2 infection is through vaccination. However, the virus had shown significant changes and mutations that resulted in the emergence of different strains. Each strain varies in its virulence, disease severity, and the body's immune system response. Previous reports showed that the Omicron variant causes mild disease. Little is known about the effect of Omicron in patients with acute leukemia. We present three patients with acute leukemia who had an infection with the Omicron variant of the SARS-CoV-2 virus.

Isolated Great Saphenous Vein Thrombosis in a Patient With COVID-19 Infection: Case Report and Review of the Literature.

On 30 January 2020, the Director-General declared that the outbreak of coronavirus disease 2019 (COVID-19) caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) constitutes a Public Health Emergency of International Concern (PHEIC), and on 11 March 2020, it was characterized as a pandemic. Since then, patients with COVID-19 infection are commonly reported to have an increased risk of thrombosis in various blood vessels due to hypercoagulability, blood stasis, and endothelial damage. In this study, we will present a case of a pregnant lady who was evaluated for right leg pain that started one week after having upper respiratory tract symptoms and COVID-19 infection confirmed by the COVID antigen (Ag) test. Further investigation with Doppler ultrasound (US) revealed complete right great saphenous vein thrombosis. This suggests that COVID-19 may lead to other adverse effects through damage to blood vessels.

The Outcome of Fatherhood in Patients With Philadelphia-Negative Myeloproliferative Neoplasms: A Single-Institution Experience.

Background Fertility is a highly complex subject; it involves more than one individual and has profound psychological and economic implications. Moreover, it is affected by several factors, including age, significant systemic illness in either partner, exposure to environmental toxins, medications, or radiation. In patients with malignancy, fertility is more complicated. Patients with a malignancy might have reduced fertility due to the disease, medication, and radiation. Besides the reduced fertility, there are more concerns regarding the subsequent effect of cancer treatment on their offspring and the possibility of having healthy children. There were many studies regarding fertility in patients with cancer; however, in male patients with Philadelphia-negative myeloproliferative neoplasms (MPNs), there are very limited data. Objectives In this study, we aim to see the outcome of fatherhood in male patients with Philadelphia-negative myeloproliferative neoplasms (MPNs), polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF) whether on treatment or not. Methods A retrospective mixed-design study of male patients with Philadelphia-negative MPN was followed up in our institute (National Center for Cancer Care and Research (NCCCR)), Doha, Qatar, between January 1, 2008, and January 1, 2020. Patients were interviewed regarding fertility-related information. All included patients had a confirmed diagnosis of Philadelphia-negative MPN according to World Health Organization (WHO) 2008 or WHO 2016 criteria for MPN, aged more than 18 years old. Results A total of 124 male patients were interviewed, and only 20 patients met the inclusion criteria. The majority of the patients were lost to follow-up or could not be contacted, and 28.8% of the excluded patients had their families completed by the time of diagnosis. The treatment received included hydroxycarbamide (n=8), pegylated interferon 2 alpha (n=10), ruxolitinib (n=1), and phlebotomy (n=1). The mean duration of exposure to treatment before pregnancy was 4.7 years. The mode of delivery was normal vaginal delivery in 71.4% of the pregnancies. The total number of offspring was 30, and the total number of conceptions was 30. Conclusion Our data showed that most Philadelphia-negative MPN male patients on treatment had their offspring born normally with no serious complications, congenital anomalies, or reports of MPN-related cancers. Patients' concerns regarding fertility should be addressed well to ensure a better quality of life.

Prevalence of neutropenia among adult Arabs in Qatar: Relation to other hematological parameters and anthropometric data.

Neutropenia ranges from a normal variant to life-threatening acquired and congenital disorders. This study aims at providing baseline information regarding the prevalence and spectrum of neutropenia in the Arab blood donors who are living in Qatar. This retrospective cohort study was conducted to review the data of healthy Arab individuals (≥18 years) who donated blood between January 1, 2015 to May 15, 2019. A complete blood count was performed using automated analyzers. The prevalence of neutropenia was 10.7%. The prevalence in females was 32% and in males, it was 6%. Absolute neutrophil count (ANC) below 1 × 109/L was detected in 10% of Arab females and 1.8 % of Arab males. In females, the neutropenic group had significantly lower hemoglobin (Hb) levels and higher red cell distribution width, and lower total white blood cells and lymphocyte counts (P < .001) compared to the group with ANC > 1.5 × 109/L. Significant correlations were found between the ANC and Hb (r = 0.33, P < .05) and ANC and total white blood cells (r = 0.45, P < .01). The prevalence of neutropenia is considerably high in Arab adult females compared to other ethnic groups. Besides the genetic constitution of Arabs, the lower Hb and higher red cell distribution width in females suggest that iron deficiency could contribute to the development of neutropenia.

Acute chest syndrome in sickle cell disease/HBE patient, A case report.

The presented case will shed some light on one of the rarest complications of HBSE disease, which is acute chest syndrome, and will highlight the management of that complication.

Use of rivaroxaban in sickle cell disease and venous thromboembolism: A case report.

INTRODUCTION: Sickle cell disease (SCD) is a hematological disorder characterized by sickling of red blood cells. Patients are at increased risk of venous thromboembolism. There are no guidelines for the management of venous thromboembolism in sickle cell disease specifically in terms of the anticoagulant of choice. PATIENT CONCERNS: Here, we report a case of a 30-year-old lady with past medical history of sickle cell disease who came with chest pain and shortness of breath. DIAGNOSIS: We found that she has bilateral pulmonary embolism (PE). INTERVENTION: She was started on rivaroxaban. OUTCOME: The patient was followed for 18 months, she did not suffer from recurrence of PE, and she did not develop any complications related to rivaroxaban. CONCLUSION: We concluded that rivaroxaban is effective in treating PE in sicklers and also it is safe.

A Rare Case of Hemoglobin E/Beta-Thalassemia and Systemic Lupus Erythematosus.

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease, with multisystemic involvement. Hemoglobin E/beta-thalassemia (HbE/beta-thalassemia) is the genotype responsible for approximately one-half of all severe beta-thalassemia worldwide. When beta-thalassemia and SLE coexist, SLE seems to have a more severe course. Here we report a 32-year-old female who presented with dizziness and fatigue was found to have severe hemolytic anemia with thrombocytopenia. Upon further evaluation, she was diagnosed with HbE/beta-thalassemia and SLE, which is a very rare association. In SLE patients, anemia usually results from the disease itself, but it is important to think of other coexisting conditions like thalassemia.