Phenotypic and Genotypic Signatures of VWF Exon 18 in Eastern Saudi Patients Previously Diagnosed with Type 1 von Willebrand Disease.

Introduction: von Willebrand disease (VWD) is the most prevalent bleeding disease, which is associated with either low levels of von Willebrand factor (VWF) or abnormality in its structure. Three types of the disease have been described; type 1 (VWD1) and 3 (VWD3) are caused by deficiency of VWF and type 2 (VWD2) is caused by production of defective VWF. The aim of the current study was to characterize gene variants of VWF gene; exon 18 in particular, in a cohort of Saudi families as well as healthy control subjects. Methods: A total of 19 families comprising 60 subjects of type 1 VWD were enrolled in the study. Participants were divided into 22 index cases, 21 affected family members and 17 unaffected family members ranging in age from 6 to 70 years. Blood samples were collected from all participants to measure activated partial thromboplastin time test (APTT), von Willebrand antigen level (VWF:Ag), Factor VIII activity (FVIII:C) and ristocetin cofactor activity (VWF:RCo), platelet count, determining the ABO blood group and for genetic analysis by Sanger sequencing. Results: The results indicated that VWD1 patients have lower levels of VWF and factor VIII than the non-affected family members and the control subjects. In addition, five gene variants were reported in VWF exon 18; of these, c.2365A>G and c.2385T>C were more common in the control group and might be protective from VWD. Discussion: In conclusion, VWF levels are influenced by blood group, and there was no association between variants in exon 18 of VWF gene reported in all groups and the disease status; however, blood group analysis and genome-wide genotyping could help to highlight high-risk groups and improve clinical management of VWD.

The in vitro generation of multi-tumor antigen-specific cytotoxic T cell clones: Candidates for leukemia adoptive immunotherapy following allogeneic stem cell transplantation.

Adoptive T-cell immunotherapy is a promising approach to manage and maintain relapse-free survival of leukemia patients, especially following allogeneic stem cell transplantation. Post-transplant adoptive immunotherapy using cytotoxic T lymphocytes (CTLs) of the donor origin provide graft-versus-tumor effects, with or without graft-versus-host disease. Myeloid leukemias express immunogenic leukemia associated antigens (LAAs); such as WT-1, PRAME, MAGE, h-TERT and others, most of them are able to induce specific T cell responses whenever associated with the proper co-stimulation. We investigated the ability of a LAA-expressing hybridoma cell line to induce CTL clones in PBMCs of HLA-matched healthy donors in vitro. The CTL clones were induced by repetitive co-culture with LAAs-expressing, HLA-A*0201(+) hybrid cell line, generated by fusion of leukemia blasts to human immortalized APC (EBV-sensitized B-lymphoblastoid cell line; HMy2). The induced cytotoxic T cell clones were phenotypically and functionally characterized by pentamer analysis, IFN-γ release ELISPOT and cellular cytotoxicity assays. All T cell lines showed robust peptide recognition and functional activity when sensitized with HLA-A*0201-restricted WT-1235-243, hTERT615-624 or PRAME100-108 peptides-pulsed T2 cells, in addition to partially HLA-matched leukemia blasts. This study demonstrates the feasibility of developing multi-tumor antigen-specific T cell lines in allogeneic PBMCs in vitro, using LAA-expressing tumor/HMy2 hybrid cell line model, for potential use in leukemia adoptive immunotherapy in partially matched donor-recipient setting.

The approach to a patient with a bleeding disorder: for the primary care physician.

Normal Hemostasis requires the interaction of platelets and the clotting cascade with normal blood vessels and supporting tissues. Bleeding problems and easy bruising are commonly encountered clinical problems. Assessment of these patients is a multistep evaluation process that involves a complete detailed history, thorough physical examination and relevant laboratory evaluation. Many disorders are usually relatively straight forward to diagnose, but in other disorders, patients may have "hidden" signs and symptoms making diagnosis more difficult. A meticulous approach must be used to plan the first steps of management.

Attitudes of medical laboratory technology graduates towards the internship training period at king faisal university.

OBJECTIVES: The objective of this present survey was to look into the attitudes of medical laboratory technology (MLT) graduates towards the internship training period of the MLT Department, College of Applied Medical Sciences, King Faisal University. MATERIAL AND METHODS: A self-administered questionnaire was designed and distributed for this purpose. The study period was from December 1(st) 2002 - 31(st) December 2004. Two-hundred questionnaires were distributed to recent graduates, and 115 were returned completed. RESULTS: All respondents agreed with the importance and necessity of the internship period, and felt it should not be reduced or eliminated. The most favorite laboratory where they liked to work was microbiology (70%). They all agreed that evaluation report with hospital staff and laboratory set up were vital in achieving the goals of the internship period. The majority stressed the significance of safety precautions and the application of theoretical knowledge before performing technical assignments. CONCLUSION: The respondents had very positive attitudes towards the internship-training period stressing its importance. The most favorite laboratory rotations were in rank order: Microbiology, Serology followed by Histotechnology, Hematology, Blood Banking and finally Clinical Chemistry. The majority of graduates had a very positive attitude also towards medical laboratory technology as a profession.

A review of predonation blood donor deferrals in a university hospital.

OBJECTIVES: To study the main causes of predonation deferral of potentially healthy prospective blood donors in a University Hospital Blood Bank unit, and to make recommendations accordingly. METHODS: A retrospective review of the main causes of predonation deferral of blood donors in King Fahd Hospital of the University (KFHU) Al-Khobar, was carried out. Records of all predonation deferrals from 1(st) January 1996 to 31(st) December 2003 were reviewed and analyzed. RESULTS: A total of 33,900 potential blood donors came to donate blood during the study period. A total of 6508 donors (19.2%) were deferred for various reasons. Analysis of the causes of deferral showed the following as the most common reasons in rank order: (1) recent ingestion or taking of counter-indicative medications; (2) low hematocrit level; (3) underweight; (4) abnormally high pulse rate; (5) low blood pressure; (6) temperature above 37.5°C; (7) High blood pressure; (8) presenting for donation too soon i.e. less than 8 weeks after the previous one; (9) age below or above the accepted limit; (10) a previous serological positive result; (11) general appearance; (12) abnormally low pulse rate. CONCLUSION AND RECOMMENDATIONS: It is important to provide donors with a clear message on their deferral status. Increased public education about blood donation and the common causes of donor deferral may lower deferral rates and prevent a negative impact on the donor himself as well as on subsequent blood donations. Public education is needed also to help recruit as many volunteer donors as possible.

Professional attitudes and career choices of female medical laboratory technology students and graduates: experience from eastern saudi arabia.

OBJECTIVES: To conduct a survey of the medical laboratory technology senior students, interns and graduates in King Faisal University to gather descriptive data on what students and graduates felt about their chosen profession and their career goals for the next five years. MATERIAL AND METHODS: The study design was a survey. A questionnaire was specially designed for this purpose in order to gather information on the professional attitudes and career choices of the participants. The study period was one year, from January I, 2004 to December 31, 2004. A stratified random sample with proportional allocation was selected and a self-administered 17-item questionnaire was distributed. A 5-point Likert scale ranging from 1, strongly agree to 5 strongly disagree was created for the questions as well as two multiple-choice questions. 115 questionnaires were distributed to the available senior students, interns and graduates, 111 were returned completed. Statistical analysis was performed using the statistical package for the social sciences (SPSS) PC + Software program. RESULTS: Respondents indicated a high level of satisfaction with their chosen profession as indicated by the ratings on the different questions relating to the profession of MLT. Their career goals were mainly working in a hospital laboratory as medical technologists (57.7%), and continuing their postgraduate education (38.7%). CONCLUSION AND RECOMMENDATIONS: MLT students and graduates expressed satisfaction with their chosen profession as evidenced by their responses assessing professional attitudes. Their career goals were mainly working at the bench level as Medical Laboratory Technologists in a hospital laboratory and postgraduate education.

Prevalence of antibodies to human T-lymphotropic viruses types I and II among healthy blood donors.

OBJECTIVE: To assess the prevalence of human T-cell lymphotropic virus type I and type II antibodies in blood donors donating blood for various reasons in a University hospital and to compare the results with other reports from the Kingdom of Saudi Arabia (KSA), and elsewhere. METHODS: A 7-year retrospective review of blood bank records for results of serological tests at the King Fahd Hospital of the University, Al-Khobar, KSA, from January 1995 to December 2001 was conducted. The study included review of blood donor questionnaire cards as well as extraction of any other relevant information. RESULTS: The results showed that the total number of blood donor units drawn during the 7- year period was 23493 units. A total of 50 units were found repeatedly reactive by enzyme immunoassay screening test (0.2%). Only 12 (0.05%) were confirmed reactive by western blot test and 4 were found to be indeterminate. Nine (0.04%) of the confirmed samples were from Saudi nationals. All 3 non-Saudi confirmed reactive donors were Indian nationals, while the 4 indeterminate cases, 2 were Saudis and 2 were Egyptians. A statistical estimate of the maximal risk of finding a positive donor in this donor population subgroup is in the order of 0.05%. The number of Saudi blood donors during this study was 16434 (80.3%) and non-Saudi donors was 4027 (19.7%). CONCLUSION: Based on these results it is shown that the prevalence of this virus is still low among blood donors in the Eastern region of Kingdom of Saudi Arabia.

Pattern of blood procurement, ordering and utilization in a University Hospital in Eastern Saudi Arabia.

OBJECTIVE: The main objectives of this study were to review blood procurement, ordering, utilization, and causes of discarding blood in a University hospital and provide recommendations for improvement. The study was also aimed at sheding light on the frequency of seropositivity for certain disease markers in blood donors. METHODS: This review comprised a retrospective 5-year analysis from January 1996 to December 2000 at King Fahad Hospital of the University, Al-Khobar, Kingdom of Saudi Arabia. RESULTS: In this study, the most common donors were replacement (46%) and statutory donors (35%), while volunteer blood donors comprised a lower percentage (19%). There was a high crossmatch transfusion ratio (2.96:1) and similarly a high percentage of cancelled transfusions after crossmatching (66.2%). The 2 most commonly ordered blood components were packed red blood cells (45.7%) and random platelet concentrates (19.2%). The infective causes for discarding blood were: hepatitis B core antibody seropositivity (16.9%), hepatitis B surface antigen seropositivity (2.4%) followed by hepatitis C antibody seropositivity (1.5%), and rapid plasma reagin (serological test for syphilis) positivity (1%). The common non-infective causes of discarding blood in descending order or frequency were: expired unit shelf life (3.6%), positive donor antibody screen (0.7%), red blood cell morphological abnormalities (0.4%), and blood unit insufficient quantity (0.3%). Release of emergency uncrossmatched blood ranged at 0.6% during the study period. CONCLUSION: Performing blood bank internal audits and reviewing statistics are vital tools for a successful blood transfusion service. Implementing policies such as type and screen and the maximum surgical blood-ordering schedules will lead to monetary savings and more effective blood utilization. Drives for enhancement of volunteer blood donors are recommended.

Bone marrow examination. Indications and diagnostic value.

OBJECTIVE: To identify the main indications for bone marrow examination in a University hospital setup and the most common diagnoses encountered. To also identify the extent of correlation, if any, between the preliminary diagnosis and the result of the final bone marrow diagnosis. METHODS: The requests and reports of all bone marrow biopsies and aspirations carried out during a 12-year period from January 1988 through to December 1999, in King Fahd Hospital of the University, Al Khobar, Kingdom of Saudi Arabia were retrospectively reviewed. The information extracted included the main indications for performing this procedure, age groups involved, and the most common diagnoses encountered. A specially designed form was used for this purpose and the data was analyzed using the statistical package for social sciences. Randomly selected slides of the most common diagnoses were reviewed to concur with the diagnosis. RESULTS: There was a total of 1813 bone marrow biopsies or aspirations, or both, performed. The main indications for bone marrow examination in a descending order of frequency were the following: The diagnosis and management of acute leukemia 403 (22.2%), staging for lymphoma 276 (15.2%), evaluation of pancytopenia 215 (11.9%), thrombocytopenia 173 (9.5%), investigation of anemia 151 (8.3%), fever (pyrexia of unknown origin) 130 (7.2%), lymphadenopathy 120 (6.6%), and hepatosplenomegaly 80 (4.4%). The most common diagnoses encountered were: acute lymphoblastic leukemia 242 (13.3%), immune thrombocytopenia 123 (6.8%), acute myeloblastic leukemia 80 (4.4%), hypersplenism 79 (4.4%), chronic granulocytic leukemia 73 (4.0%), megaloblastic anemia 66 (3.6%), bone marrow positive for lymphomatous infiltration 63 (3.5%), chronic lymphocytic leukemia 40 (2.2%), and multiple myeloma 32 (1.8%). CONCLUSION: This study confirms that bone marrow examination is a very important investigation for establishing the diagnosis in many conditions, especially hematological neoplasms. The most common indication for this procedure in our hospital was to confirm and manage acute leukaemia and the most common diagnosis was acute lymphoblastic leukemia.

Medical laboratory technology program at king faisal university: a 10-year experience.

This paper documents the evolution of the Medical Laboratory Technology (MLT) program established in 1989 (1408/1409 H) at the College of Medicine and Medical Sciences, King Faisal University. The rationale, objectives, the general outline of the program as well as methods of instruction and evaluation are discussed. The internship period and future plans are also addressed. Two hundred and seventy (270) students had been enrolled in the program since its inception until September 2000. Ten batches (138 graduates) have already successfully graduated. One hundred and fifteen (83.3%) graduated technologists are employed in the different health sectors and educational institutions in the Kingdom.