Von Willebrand disease (vWD) is an inherited bleeding disorder caused by deficiency or dysfunction of von Willebrand factor (vWF). The disease is relatively common. Patients commonly visit primary care, general pediatricians, and general internist physicians; therefore, these practitioners require education and guidance to treat patients with vWD. Here, we present guidelines designed to meet the needs and special conditions for healthcare practitioners in Saudi Arabia. Keywords: von Willebrand Disease, guidelines, desmopressin, von Willebrand factor, diagnosis, management

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Despite advances in the treatment of acute lymphoblastic leukemia (ALL), the etiology of the disease remains obscure. Various theories based on patient demographics have been proposed to explain its unique incidence pattern. Given this approach, epidemiological data collected from different countries would be useful for elucidating the important predisposing and precipitating factors for ALL. This study was undertaken to collect, analyze, and correlate morphologic, cytochemical, immunophenotypic, and cytogenetic data from patients diagnosed with ALL at an Indian specialty cancer center. Morphological data was recorded after staining with Leishman or May-Grόnwald-Giemsa stains. Lineage assignment was performed using 3-colour flow cytometry and a primary panel of lineage- specific antibodies. For cytogenetic studies, short-term cultures of sample cells were arrested using colcemid and G-banded using trypsin and Giemsa stain. A total of 61 patients were evaluated, of which 41 had B-lineage ALL and 20 had T-lineage ALL. Expression of myeloid antigens was the most common aberrant feature seen and was usually associated with the expression of early embryonic markers. A patient with mixed-phenotype ALL, i.e., with both T and B markers was observed. Karyotyping generally supported the immunophenotyping results, but also identified rare cases, such as del(5q)-ALL. Epidemiological studies of ALL in developing countries in conjunction with flow cytometric lineage analysis, karyotyping, and clinical follow-up would be of great help in building a better understanding of the causal factors of ALL.

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Deletion of 6q21 and amplification of 8q24 (c-Myc) are associated with poor prognosis in lymphoproliferative disorders and may identify patients with poor prognosis. In this study, we evaluated patients with chronic lymphocytic leukemia (CLL) for common and less common genetic aberrations. We correlated the 6q21 and c-Myc aberrations with standard prognostic markers and clinical outcomes of CLL. We used the fluorescence in situ hybridization (FISH) technique to detect the 6q21 deletion and c-Myc amplification simultaneously in 75 patients with B-cell CLL (B-CLL). Additionally, we used a routine panel of probes (locus-specific identifier [LSI] 13q14 and 17p13, and centromere 12) together with flow cytometry using the routine panel for lymphoproliferative disorder. We detected the 6q21 deletion in 14 of 75 patients (18.7%) and the c-Myc amplification in 20 patients (26.7%). Each aberration associated with a shorter period of disease-free survival. The 6q21 deletion associated significantly with low hemoglobin (Hb), high total leucocytes, low platelet counts, short lymphocyte doubling time (LDT), advanced Rai clinical staging, and increased CD38 expression. The amplification of c-Myc was associated significantly with low Hb, increased total leukocyte count (TLC), and short LDT. Deletions in 13q14 occurred in 18 patients (24%), trisomy 12 occurred in 3 (4%), and deletions in 17q (p53) occurred in 2 (2.7%). Disease outcomes revealed significant associations between several standard prognostic markers, including old age, sex, advanced Rai staging, LDT, and CD38 expression. In conclusion, the detection of 6q21 deletion and c-Myc amplification in CLL patients indicates poor prognosis and may influence therapeutic decisions; these cytogenetic aberrations warrant further investigation.

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Myasthenia gravis crisis (MGC) is a life-threatening condition that can complicate the course of MG. Some case reports suggest the value of rituximab, a B-cell monoclonal antibody, in refractory cases. We report the case of a 54-year-old woman with progressive AChR+ myasthenia gravis (MG) and recurrent MGC that eventually required long-term mechanical ventilation. One cycle of rituximab resulted in successful weaning from mechanical ventilation and an improvement of the MG composite test score. The favorable response to rituximab indicates that rituximab can be used for refractory MG patients receiving long-term ventilation to facilitate weaning from mechanical ventilation.

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We report a case of severe sudden massive intra-abdominal hemorrhage in a patient with Glanzmann's thrombasthenia secondary to a ruptured ovarian (luteal) cyst that was successfully treated with platelet transfusion, recombinant factor VIIa, tranexamic acid, as well as packed red blood cell transfusion. After approximately 10 days of aggressive hemostatic therapy, the patient was successfully discharged and was advised to indefinitely continue administration of oral contraceptive pills.

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Integrated problem-based learning (PBL) is widely implemented in medical curricula. King Saud Bin Abdulaziz University for Health Sciences College of Medicine (KSAU-HS COM), Saudi Arabia, has adopted a graduate-entry system-based integrated PBL curriculum. PBL as a teaching method is used in the hematopoietic system block, consisting of 5 problems over 6 weeks. The aim of this study was to describe the design, implementation, and evaluation of this block as applied in KSAU-HS. Additionally, this paper aimed to reflect on student satisfaction with the block in terms of perception, relevance, and the amount of knowledge gained from it. Drawing on his experience as coordinator of the hematopoietic system block for the past 6 years, the author describes the design and implementation of this block, and reviews all existing records related to it. To determine student perceptions, a questionnaire was designed and distributed to 4 batches of firstyear medical students at the end of the block over 2009-2012. A Likert-type rating scale and open-ended answer questionnaire was used to explore both the extent and forms of students (n = 130) in teaching and learning. The data were collected and analyzed to determine the median and to measure qualitative differences. Over the 4 years, 130 questionnaires (response rate 62.5%) were collected. Hematopoietic PBL scenario rating scores indicated that students perceived the amount of knowledge learned, stimulation of creative thinking, and PBL relevance to their socio-cultural backgrounds favorably. The study revealed the students' high satisfaction with the block and provided some guidelines to shape further development of an integrated PBL in the KSAU-HS COM.

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