Saudi expert consensus on acquired hemophilia A diagnosis and management.

Objectives: Acquired hemophilia affects approximately one in 1 million people. Timely diagnosis is key to appropriate disease management and the prevention of life-threatening complications. Patients with this condition may initially be seen by inexperienced physicians and remain underdiagnosed for several years.

Clinical and laboratory presentation of von Willebrand disease: Experience from a single center in Saudi Arabia.

Objectives: This study was aimed at assessing the clinical presentations and laboratory findings among patients diagnosed with vWD at a Saudi tertiary care unit.

Methods: This retrospective study included 189 patients with vWD who were followed up in our unit over 4 years. Clinical and laboratory data were collected and analyzed in SPSS.

Outcomes of Left Main Revascularization in Patients with Anemia: Gulf Left Main Registry.

Introduction: The aim of this study was to evaluate the effects of baseline anemia and anemia following revascularization on outcomes in patients with unprotected left main coronary artery (ULMCA) disease.

Methods: This was a retrospective, multicenter, observational study conducted between January 2015 and December 2019. The data on patients with ULMCA who underwent revascularization through percutaneous coronary intervention (PCI) or coronary artery bypass graft (CABG) were stratified by the hemoglobin level at baseline into anemic and non-anemic groups to compare in-hospital events.

Pregnancy with Paroxysmal Nocturnal Hemoglobinuria: A Case Series with Review of the Literature.

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired hematopoietic stem cell disorder, and eculizumab and ravulizumab are its two approved therapies. Only few case series/reports have reported the outcomes of pregnancies in patients with PNH despite the increased risk of thrombosis. Similarly, there is limited knowledge regarding the effect of the approved treatments on conception and pregnancy outcomes.

Safety and Efficacy of Convalescent Plasma for Severe COVID-19: Interim Report of a Multicenter Phase II Study from Saudi Arabia.

Objective: To present the interim findings from a national study investigating the safety and efficacy of convalescent plasma (CP) containing detectable IgG antibodies as a treatment strategy for severe coronavirus disease 2019 (COVID-19).

Trial Design And Participants: An open label, two-arm, phase-II clinical trial conducted across 22 hospitals from Saudi Arabia. The intervention group included 40 adults (aged ≥18 years) with confirmed severe COVID-19 and the control group included 124 patients matched using propensity score for age, gender, intubation status, and history of diabetes and/or hypertension.

Highlights of the Management of Adult Histiocytic Disorders: Langerhans Cell Histiocytosis, Erdheim-Chester Disease, Rosai-Dorfman Disease, and Hemophagocytic Lymphohistiocytosis.

Histiocytic disorders are an exceptionally rare group of diseases with diverse manifestations and a paucity of approved treatments, thereby leading to various challenges in their diagnosis and management. With the discovery of novel molecular targets and the incorporation of targeted agents in the management of various adult histiocytic disorders, their management has become increasingly complex. In an attempt to improve the understanding of the clinical features and management of common adult histiocytic disorders (Langerhans cell histiocytosis, Erdheim-Chester disease, Rosai-Dorfman disease, and hemophagocytic lymphohistiocytosis), we created this document based on existing literature and expert opinion.

Molecular yield of targeted sequencing for Glanzmann thrombasthenia patients.

Glanzmann thrombasthenia (GT) is a rare autosomal recessive bleeding disorder. Around 490 mutations in and genes were reported. We aimed to use targeted next-generation sequencing (NGS) to identify variants in patients with GT.

Alemtuzumab-containing reduced intensity conditioning allogenic hematopoietic stem cell transplantation in a case of primary progressive multiple sclerosis.

Increasing evidence has emerged lately regarding the use of autologous hematopoietic stem cell transplantation (HSCT) in the treatment of aggressive multiple sclerosis (MS). However, data is scarce regarding the use of allogenic HSCT in treating MS. We present a 42 years old male with aplastic anemia who underwent allogenic HSCT for severe aplastic anemia.

Recurrent venous thrombosis in a patient with Ebstein's anomaly.

Herein, we report a case of a 27-year-old man with Ebstein's anomaly and a history of unexplained recurrent venous thrombosis despite adequate anticoagulation. After surgical correction of the Ebstein's anomaly, the venous thromboembolic events did not recur. This case demonstrates the possible etiopathogenesis of Ebstein's anomaly in causing recurrent venous thromboembolism, which is likely caused through impedance of venous blood flow.

Hematopoietic Stem Cell Transplant in Adolescent and Young Adults With Fanconi Anemia Is Feasible With Acceptable Toxicity, With Those Surviving 100 Days Posttransplant Having Excellent Outcomes.

Objectives: Fanconi anemia is a congenital bone marrow failure syndrome that is associated with congenital anomalies and increased risk of cancer. Hematopoietic stem cell transplant is a potentially curative modality for bone marrow failure in Fanconi anemia patients. Here, we report our center's experience regarding adolescent and young adult patients with Fanconi anemia and hematopoietic stem cell transplant.

Chimerism Analysis of Cell-Free DNA in Patients Treated with Hematopoietic Stem Cell Transplantation May Predict Early Relapse in Patients with Hematologic Malignancies.

Background. We studied DNA chimerism in cell-free DNA (cfDNA) in patients treated with HSCT. Methods.

Leukemia during pregnancy: long term follow up of 32 cases from a single institution.

Background And Objectives: There is limited information regarding the outcome of patients treated for leukemia during pregnancy. This study was performed on all cases of leukemia during pregnancy identified in our institution leukemia database.

Patients And Methods: It is a retrospective study from our existing database.

Allogeneic hematopoietic stem cell transplantation in adolescent and adult patients with high-risk T cell acute lymphoblastic leukemia.

Allogeneic hematopoietic stem cell transplantation (allo-SCT) is often recommended for patients with T cell acute lymphoblastic leukemia (T-ALL) in second or later complete remission (≥CR2) and sometimes in high-risk (HR) patients in first complete remission (CR1). Between January 1995 and July 2009, 53 patients with HR T-ALL underwent allo-SCT at our institution. Median age was 18 years (range, 14-51).

Klebsiella oxytoca bacteremia causing septic shock in recipients of hematopoietic stem cell transplant: Two case reports.

Background: Klebsiella oxytoca can cause various infectious complications in healthy as well as in immunocompromised individuals.

Case Presentations: Case 1: A 49 year old female with multiple myeloma received an autologous hematopoietic stem cell transplant in October 2005. Eight days following her autograft she developed septic shock caused by Klebsiella oxytoca bacteremia which was successfully treated with intravenous meropenem and gentamicin.

Successful outcome of Langerhans cell histiocytosis complicated by therapy-related myelodysplasia and acute myeloid leukemia: a case report.

Background: Various therapeutic options are available for the management of Langerhans cell histiocytosis. However, treatment administered to control this disease may be complicated by acute leukemia.

Case Presentation: A 34 years old male was diagnosed to have Langerhans cell histiocytosis in March 1999.

The Wilms' tumor antigen is a novel target for human CD4+ regulatory T cells: implications for immunotherapy.

Compelling evidences indicate a key role for regulatory T cells (T(reg)) on the host response to cancer. The Wilms' tumor antigen (WT1) is overexpressed in several human leukemias and thus considered as promising target for development of leukemia vaccine. However, recent studies indicated that the generation of effective WT1-specific cytotoxic T cells can be largely affected by the presence of T(regs).