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.

Response to Lisinopril in Patients with Sickle Cell Anemia and Proteinuria.

Proteinuria is a manifestation of sickle cell anemia (SCA)-related renal disease and is a risk factor of renal impairment. Angiotensin-converting enzyme (ACE) inhibitors have benefits, but their role in SCA remains undefined. This study aimed to assess the role of lisinopril, an ACE inhibitor, in reducing proteinuria in SCA patients.

Prevalence of bleeding symptoms among young adults in Saudi Arabia, a national survey.

Prevalence of bleeding disorders vary due to several factors including geographical location. Mild bleeding disorders can lead to iron deficiency, morbidity, and in severe cases mortality. Quantification of haemorrhagic symptoms is a key component in management of bleeding disorders and a challenging task for clinicians.

Frequency of Rh and K antigens in blood donors in Riyadh.

Objectives: Investigate the prevalence of Rh and the K antigens and their phenotypes in the red blood cells of blood donors in Riyadh, Saudi Arabia.

Methods: This is a retrospective study. The five principal Rh antigens (D, C, c, E, e) and the Kell antigen from the Kell blood group were tested in 4,675 random samples collected from four blood bank centers in Riyadh.

Iron Deficiency and Iron Deficiency Anemia Are Common Epidemiological Conditions in Saudi Arabia: Report of the National Epidemiological Survey.

Iron deficiency is the most prevalent nutritional deficiency worldwide. According to an estimate by the World Health Organization, up to 27% of the world's population experience iron deficiency anemia (IDA). Studies conducted in the Middle East, including Saudi Arabia, have suggested that IDA is the most common cause of anemia, especially among females.

Effect of Poloxamer 188 vs Placebo on Painful Vaso-Occlusive Episodes in Children and Adults With Sickle Cell Disease: A Randomized Clinical Trial.

Importance: Although effective agents are available to prevent painful vaso-occlusive episodes of sickle cell disease (SCD), there are no disease-modifying therapies for ongoing painful vaso-occlusive episodes; treatment remains supportive. A previous phase 3 trial of poloxamer 188 reported shortened duration of painful vaso-occlusive episodes in SCD, particularly in children and participants treated with hydroxyurea.

Objective: To reassess the efficacy of poloxamer 188 for vaso-occlusive episodes.

Fibrinogen concentrate for treatment of bleeding and surgical prophylaxis in congenital fibrinogen deficiency patients.

Background: Congenital fibrinogen deficiency is an ultra-rare disorder in which patients can experience severe and/or frequent bleeding episodes (BEs). Here, we present the largest prospective study to date on the treatment of this disorder.

Methods: Hemostatic efficacy of human fibrinogen concentrate (HFC; FIBRYGA , Octapharma AG) for treatment of bleeding or surgical prophylaxis was assessed by investigators and adjudicated by an independent data monitoring and endpoint adjudication committee (IDMEAC) according to a four-point scale, using objective criteria.

Impact of Day 14 Bone Marrow Biopsy on Re-Induction Decisions and Prediction of a Complete Response in Acute Myeloid Leukemia Cases.

Background: With acute myeloid leukemia (AML), there are limited data about the accuracy of day 14 bone marrow (BM) biopsies for predicting complete remission as compared to day 28 BM biopsy results. We here aimed to estimate the correlation between, and the diagnostic accuracy of, both approaches. Materials and Methods: We reviewed 84 patients with AML treated with standard induction chemotherapy to evaluate the remission rate and treatment decisions based on day 14 BM biopsy from 2000-2012.

Influence of ABO blood group on von Willebrand factor tests in healthy Saudi blood donors.

: Von Willebrand disease is a common bleeding disorder. The wide variation in von Willebrand factor (VWF) levels between and within normal individuals highlights the clinical challenge of defining its cutoff value. Although studies on the influence of ethnicity on ABO phenotypes and the levels of VWF have been carried out on different ethnicities, there is a lack of such data among Arab population.

Efficacy and safety of a new human fibrinogen concentrate in patients with congenital fibrinogen deficiency: an interim analysis of a Phase III trial.

Background: Fibrinogen concentrate is the preferred choice for fibrinogen replacement in congenital fibrinogen deficiency. This study investigated hemostatic efficacy of a new plasma-derived, double virus-inactivated (using two dedicated virus inactivation/elimination steps) human fibrinogen concentrate for on-demand treatment of bleeding episodes (BEs) and surgical prophylaxis.

Study Design And Methods: In this planned interim analysis of a prospective, multinational Phase III study (NCT02267226), 13 patients with afibrinogenemia (≥12 years) received fibrinogen concentrate (FIBRYGA, Octapharma AG).

A comparison of two techniques of preparing bone marrow aspirate slides.

Objective: To compare direct smear technique with ethylenediaminetetraacetic acid (EDTA) preserved smear technique in terms of preparing bone marrow aspirate slides.

Methods: This prospective study was carried out between September 2009 and July 2012 at the Haematology/Oncology Department, King Khalid University Hospital, Riyadh, Saudi Arabia. With a standard gauge disposable bone marrow aspirate needle, 0.

Home treatment of haemarthrosis with recombinant activated factor VII in patients with haemophilia A or B and inhibitors: experience from developing countries.

Home therapy for uncomplicated mild/moderate bleeding can decrease healthcare burden, promote self-esteem, reduce complications, and provide near-normal quality of life. To evaluate recombinant activated factor VII (rFVIIa) as home therapy for joint bleeds in Algeria, Morocco, Oman, Saudi Arabia, and United Arab Emirates. Twenty-seven patients aged more than 2 years with congenital haemophilia and inhibitors were monitored for up to 8 months after a first haemarthrosis episode treated with rFVIIa.

Lactate dehydrogenase as a biomarker for early renal damage in patients with sickle cell disease.

Among many complications of sickle cell disease, renal failure is the main contributor to early mortality. It is present in up to 21% of patients with sickle cell disease. Although screening for microalbuminuria and proteinuria is the current acceptable practice to detect and follow renal damage in patients with sickle cell disease, there is a crucial need for other, more sensitive biomarkers.

Unusual presentation of arsenic poisoning in a case of celiac disease.

Arsenic poisoning may occur from sources other than drinking water such as rice, seafood, or insecticides. Symptoms and signs can be insidious, non-specific, atypical, and easily overlooked. We present a 39-year-old woman with celiac disease who was on gluten-free diet for 8 years and presented with diarrhea, headache, insomnia, loss of appetite, abnormal taste, and impaired short-term memory and concentration, but with no skin lesions.

Immunological evaluation of β-thalassemia major patients receiving oral iron chelator deferasirox.

Objective: To determine the immune abnormalities and occurrence of infections in transfusion-dependent β-thalassemia major patients receiving oral iron chelator deferasirox (DFX).

Study Design: An observational study.

Place And Duration Of Study: Hematology Clinics, King Khalid University Hospital, Riyadh, Saudi Arabia, from July to December 2010.

Intrapulmonary rFVIIa for life threatening pulmonary hemorrhage in a case of relapsing acute lymphoblastic leukemia and platelet refractoriness.

Platelet refractoriness may lead to life-threatening gastro-intestinal, intracranial or pulmonary hemorrhage that is difficult to control despite massive platelet and red cell transfusion, antifibrinolytic agents, high dose corticosteroids, immunoglobulin and intravenous (I.V.) recombinant activated factor VII (rFVIIa).

Regional consensus opinion for the management of Beta thalassemia major in the Arabian Gulf area.

Thalassemia syndrome has diverse clinical presentations and a global spread that has far exceeded the classical Mediterranean basin where the mutations arose. The mutations that give rise to either alpha or beta thalassemia are numerous, resulting in a wide spectrum of clinical severity ranging from carrier state to life-threatening, inherited hemolytic anemia that requires regular blood transfusion. Beta thalassemia major constitutes a remarkable challenge to health care providers.

Clofarabine in the treatment of elderly patients with acute myeloid leukemia.

Background: Elderly patients with acute myeloid leukemia (AML) have a poor outcome because of co- morbidities, poor tolerance to intensive chemotherapy and inherently more resistant disease. Clofarabine is a second generation nucleoside analogue which has shown promising activity in elderly patients with AML. This study was conducted to review the outcome of treatment with clofarabine in a group of such patients.

LPS-responsive beige-like anchor (LRBA) gene mutation in a family with inflammatory bowel disease and combined immunodeficiency.

Background: Clinical immunology has traditionally relied on accurate phenotyping of the patient's immune dysfunction for the identification of a candidate gene or genes for sequencing and molecular confirmation. Although this is also true for other branches of medicine, the marked variability in immune-related phenotypes and the highly complex network of molecules that confer normal host immunity are challenges that clinical immunologists often face in their quest to establish a specific genetic diagnosis.

Objective: We sought to identify the underlying genetic cause in a consanguineous family with chronic inflammatory bowel disease-like disorder and combined immunodeficiency.

Heparin-induced thrombocytopenia: comparison between response to fondaparinux and lepirudin.

Background: Patients with heparin-induced thrombocytopenia (HIT) require anticoagulation with alternative immediate acting anticoagulants such as lepirudin. Lepirudin may generate antibodies that increase risk of bleeding. Fondaparinux, on the other hand, is structurally too short to induce antibody formation, and therefore, it could be a useful agent for the treatment of HIT.