Bleeding symptoms in patients diagnosed as type 3 von Willebrand disease : Results from 3WINTERS-IPS, an international and collaborative cross-sectional study

Background Type 3 von Willebrand's disease (VWD) patients present markedly reduced levels of von Willebrand factor and factor VIII. Because of its rarity, the bleeding phenotype of type 3 VWD is poorly described, as compared to type 1 VWD. Aims To evaluate the frequency and the severity of bleeding symptoms across age and sex groups in type 3 patients and to compare these with those observed in type 1 VWD patients to investigate any possible clustering of bleeding symptoms within type 3 patients. Methods We compared the bleeding phenotype and computed the bleeding score (BS) using the MCMDM-1VWD bleeding questionnaire in patients enrolled in the 3WINTERS-IPS and MCMDM-1VWD studies. Results In 223 unrelated type 3 VWD patients, both the BS and the number of clinically relevant bleeding symptoms were increased in type 3 as compared to type 1 VWD patients (15 versus 6 and 5 versus 3). Intracranial bleeding, oral cavity, hemarthroses, and deep hematomas were at least five-fold over-represented in type 3 VWD. A more severe bleeding phenotype was evident in patients having von Willebrand factor antigen levels <20 IU/dL at diagnosis in the two merged cohorts. In type 3 patients, there was an apparent clustering of hemarthrosis with gastrointestinal bleeding and epistaxis, whereas bleeding after surgery or tooth extraction clusters with oral bleeding and menorrhagia. Conclusions In the largest cohort of type 3 VWD patients, we were able to describe a distinct clinical phenotype that is associated with the presence of a more severe hemostatic defect.Peer reviewe

Evaluation of Staphylococcus Aureus and Pseudomonas Aeruginosa in Saliva of Patients with Acute Myeloid Leukemia

Statement of the Problem: Patients with leukemia are prone to infectious and often life-threatening complications. Evidence suggests that a specific oral microbiota may contribute to septicemia, which can delay antineoplastic treatment, compromise treatment efficacy, or even endanger patients' livesPurpose: This study investigated the prevalence of Staphylococcus aureus and Pseudomonas aeruginosa in the saliva of patients with acute myeloid leukemia who were candidates for bone marrow transplantation.Materials and Method: This cross-sectional study was conducted in 2019 in the Hematology-Oncology Department of Namazi Hospital, Shiraz University of Medical Sciences. The study included 28 patients with acute myeloid leukemia eligible for bone marrow transplantation as the case group and age- and sex-matched healthy individuals as the control group. Unstimulated saliva samples were collected to determine the frequency of Staphylococcus aureus and Pseudomonas aeruginosa. Data were analyzed using SPSS version 18, the chi-square test, and the independent t-test.Results: In the patients with acute myeloid leukemia, 26 (86%) were positive for Staph-ylococcus aureus and 18 (60%) were positive for Pseudomonas aeruginosa. In the healthy group, 11 (22.9%) were positive for Staphylococcus aureus and 3 (6.2%) were positive for Pseudomonas aeruginosa. The frequency of Pseudomonas aeruginosa and Staphylococcus aureus in the saliva samples of patients with acute myeloid leukemia was significantly higher than in the healthy control subjects (p value < 0.05). Chi-square test showed no significant association between age and the frequency of bacteria (p value= 0.27).Conclusion: In the current study, the frequency of Staphylococcus aureus and Pseudomonas aeruginosa in the saliva of patients with acute myeloid leukemia was higher than in the healthy control group

Complementary and alternative medicine use in thalassemia patients in Shiraz, southern Iran: A cross-sectional study

This study aimed to determine the frequency and pattern of complementary and alternative medicine (CAM) use in thalassemia patients in south of Iran. The survey was done using a validated questionnaire which was distributed among 122 thalassemia patients. Only 108 questionnaires were completed and turned back (response rate 88.5%). Patients referred to an outpatient thalassemia clinic in Shiraz, southern Iran for blood transfusion. The mean age of the patients was 22.9 ± 7.9 years (range 4–45 years) with female/male ratio 1.84. Seventy four (68.5%) of the responders used CAM at least once during their life, and about half of them used it concurrently with their conventional treatments. The most reported CAM product was mint juice (50%). The most common reason of CAM use was increased general health. The most common information source about CAM was physicians who were the most trusted source as well. CAM is frequently being used in thalassemia patients to ensure their sense of well-being and help them overcome the complications of their illnesses

Potential voriconazole associated posterior reversible leukoencephalopathy in children with malignancies: Report of two cases

The fungal infection has become severe morbidity amongst patients with malignancy. Voriconazole, a new generation of triazole, has shown excellent results in treating invasive fungal infections. Herein, we report two cases of posterior reversible encephalopathy syndrome (PRES), which induced after voriconazole exposure. Magnetic resonance imaging, and the serum level of voriconazole were investigated in both patients to assess toxicity. The role of methotrexate, as one of the possible causes of PRES, is weakened significantly through precise assessing diffusion-weighted images on magnetic resonance imaging. These unique cases emphasize that voriconazole can induce PRES even at therapeutic levels. Therefore, in the case of neurotoxicity, PRES must be considered, and voriconazole should discontinue. The prognosis seemed promising when voriconazole stopped immediately after clinical suspicion