findings provide further evidence for a high prevalence of VTE in critically ill patients with SARS-CoV-2 infection. These findings are important for identifying a high risk group for adverse outcomes and to raise clinicians' awareness of VTE risk amongst critically ill COVID-19 patients.

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REFERENCES 1. Ljung RC. Intracranial haemorrhage in haemophilia A and B. Br J Haematol. 2008;140:378-384. 2. Witmer C, Presley R, Kulkarni R, et al. Associations between intracranial haemorrhage and prescribed prophylaxis in a large cohort of haemophilia patients in the United States. Br J Haematol. 2011;152:211-216. 3. Nelson MD Jr, Maeder MA, Usner D, et al. Prevalence and incidence of intracranial haemorrhage in a population of children with haemophilia. The Hemophilia Growth and Development Study. Haemophilia. 1999;5:306-312. 4. Morrison L, Akers A. Cerebral Cavernous Malformation, Familial. 1993. 5. McCormick WF. Pathology of vascular malformations of the brain. In: Wilson CB, Stein BM eds. Intracranial Arteriovenous Malformations. Baltimore: William & Wilkins, 1984: 44. 6. Lanfranconi S, Ronchi D, Ahmed N, et al. A novel CCM1 mutation associated with multiple cerebral and vertebral cavernous malformations. BMC Neurol. 2014;14:158. 7. Denier C, Labauge P, Brunereau L, et al. Clinical features of cerebral cavernous malformations patients with KRIT1 mutations. Ann Neurol. 2004;55:213-220. 8. Zabramski JM, Wascher TM, Spetzler RF, et al. The natural history of familial cavernous malformations: results of an ongoing study. J Neurosurg. 1994;80:422-432. 9. Akers A, Al-Shahi Salman R, A Awad I, et al. Synopsis of guidelines for the clinical management of cerebral cavernous malformations: consensus recommendations based on systematic literature review by the angioma alliance scientific advisory board clinical experts panel. Neurosurgery. 2017;80:665-680. 10. Riley RR, Witkop M, Hellman E, Akins S. Assessment and management of pain in haemophilia patients. Afibrinogenemia is a rare bleeding disorder, which usually presents with abnormal umbilical cord bleeding or spontaneous bleeding requiring fibrinogen concentrate, or cryoprecipitate substitution therapy. Its frequency is estimated 1 per 1 million normal population with autosomal recessive inheritance pattern. 1,2 Data on treatment of patients with afibrinogenemia during pregnancy and delivery are scarce, and there is no evidence-based guideline for treatment of these patients during pregnancy. Most data are based on personal experience from around the world. 3-7We report a case of successful delivery in a patient with afibrinogenemia after 3 consecutive abortions. In addition, we review the available literature treating pregnant women with afibrinogenemia.A 23-year-old woman who presented at the age of 5 years old with postdental extraction bleeding was diagnosed as afibrinogenemia (fibrinogen level was <35 mg·dL −1 and her genetic analysis showed a homozygote with a new nonsense premature stop codon mutation [g.4347G>T; Gly358Stop]).She had aborted twice at gestational age of 5 weeks prior to taking replacement prophylactic therapy. She received replacement therapy starting with 1 g per week at gestational age of 7 weeks during her third pregnancy maintaining fibrinogen level of 77 mg·dL −1 . She was hospitalized due to vaginal bleeding at gestational age of 17 ...

We evaluated the correlation between thrombin generation (TG) parameters with bleeding symptoms and disease severity in patients with hemophilia. In this cross-sectional study, 59 patients with hemophilia without inhibitors and regardless of their severity were randomly selected from southern Iran and TG assays were conducted. Bleeding score (BS) was calculated by performing a clinical evaluation using Tosetto questionnaire. Only lag time showed a statistically significant correlation with BS (rs = .316,P= .016). All TG parameters except peak showed association with disease severity (P< .05). Endogenous thrombin potential showed a significant correlation with factor activity level (rs = .459,P< .001). Both lag time and start tail showed significant negative correlations with factor activity level (rs = -0.488,P< .001 andrs = - .289,P< .026, respectively). Although most of the TG parameters evaluated were not significantly correlated with the BS of patients with hemophilia, the majority of TG parameters were significantly associated with factor activity level and disease severity.

Background:  The ongoing COVID-19 pandemic, caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has led to high morbidity and mortality worldwide. Vaccination against SARS-CoV-2 is a leading strategy to change the course of the COVID-19 pandemic. Aims of study: Our aim was to investigate the efficacy and side effects of Sinopharm vaccine in patients with hemoglobinopathies in Iran, and the frequency of breakthrough infection after a full   course of vaccination. Methods: A multicenter cross-sectional study of 434 patients with hemoglobinopathies (303 β-thalassemia major, 118 β-thalassemia intermedia, and 13 sickle-thalassemia) were conducted from March to July 2021 in IRAN. All patients have received the first dose of China Sinopharm vaccine and received the second dose of the vaccine 28 days apart. Antibody testing: Detection of immunity after vaccination was evaluated by commercial enzyme- linked immunosorbent assay (Pishtazteb ELISA commercial kit), including a surrogate virus neutralization test (sVNT), for detection of SARS-CoV-2 immunoglobulins (IgA, IgM, IgG), total neutralizing antibody (NAb). Results: The mean age of patients was 35.0± 8.5 (from 18 to 70) years and 55.6% were positive for antibody. Overall, 48.2% of the studied population had at least one side effect after vaccination. The most frequent side effects were fever and chills, dizziness, and body pain. A total of 90 (20.7%) vaccinated patients developed breakthrough infections after full Sinopharm vaccination. Disease severity was recorded, and it was classified as mild in 77.8%, moderate in 13.6%, and severe in 7.4% of patients. One 28-year-old woman with β- thalassemia major died eight days after the diagnosis of breakthrough SARS-CoV-2 infection. Discussion: No safety concerns were identified in patients who received two doses of Sinopharm vaccine. Its efficacy was not optimal which might be due to lack of its effect on new variations of virus. However, our data show that it can prevent from severity of COVID-19 infection in patients with hemoglobinopathies. The frequency of breakthrough infections after full Sinopharm vaccination support the evolving dynamic of SARS-CoV-2 variants requiring special challenge, since such infection may represent a risk for vulnerable patients.