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Patients with Sticky Platelet Syndrome, Sickle Cell Disease And MAEDICA – a Journal of Clinical Medicine Mædica - a Journal of Clinical Medicine 2021; 16(2): 268-273 https://doi.org/10.26574/maedica.2021.16.2.268 REVIEW Patients with Sticky Platelet Syndrome, Sickle Cell Disease and Glanzmann Syndrome May Promulgate Severe Thrombosis if Infected with Covid-19 Nitin Ashok JOHNa, Jyoti JOHNb, Praful KAMBLECa, Anish SINGHALa, Vandana DAULATABADc, I. S. VAMSHIDHARa aDepartment of Physiology, All India Institute of Medical Sciences, Bibinagar, Telangana, India bDepartment of Biochemistry, All India Institute of Medical Sciences, Nagpur, Maharashtra, India cDepartment of Physiology, Dr. V. M. Government Medical College, Solapur, Maharashtra, India ABSTRACT Covid-19 pandemic has been a very serious cause of health concern worldwide. Thrombosis has been a critical manifestation in severe Covid-19 infection. The increased arterial and venous thrombosis in patients with Covid-19 is proving to be life threatening. Sticky platelet syndrome and sickle cell disease are genetic disorders with procoagulant nature of the disease, while in Glanzmann syndrome there is an enhanced bleeding tendency, with pathological defect leading to altered platelet aggregation and delayed clot formation. Considering the thrombotic episodes of Covid-19, we decided to review the literature on data bases such as PubMed and Medline for knowing the coagulant status in genetically associated diseases such as sticky platelet syndrome, sickle cell disease and Glanzmann syndrome. We planned to review various published studies with the aim to find whether the coagulant profiles in these conditions alter the thrombotic manifestations and prognosis if these patients contract Covid-19. Various research studies revealed that patients with sticky platelet syndrome develop arterial and venous thrombosis, while those with sickle cell disease are known to develop complications such as deep vein thrombosis and pulmonary embolism. Moreover, patients with Glanzmann syndrome who usually have a bleeding tendency also rarely present with severe venous and arterial thrombosis and pulmonary embolism. Patients with sticky platelet syndrome and sickle cell disease and,, occasionally those with Glanzmann syndrome have a higher risk for thrombosis if infected with Covid-19. More studies are needed to better understand the clinical manifestations and designing standard management protocol for patients with sticky platelet syndrome, sickle cell disease and Glanzmann syndrome who contract Covid-19 infections. Keywords: Sticky platelet syndrome, sickle cell disease, Glanzmann syndrome, thrombosis, pulmonary embolism, Covid-19. Address for correspondence: Dr. Nitin Ashok John, MD, Professor & Head Department of Physiology, All India Institute of Medical Sciences, Bibinagar, Telangana, 508126, India Email: [email protected] Article received on the 1st of May 2021 and accepted for publication on the 27th of May 2021 268 Maedica A Journal of Clinical Medicine, Volume 16, No. 2, 2021 STICKY PLATELET SYNDROME, SICKLE CELL DISEASE AND GLANZMANN SYNDROME, AND SEVERE THROMBOSIS WITH COVID-19 INTRODUCTION tations and prognosis if these patients contracted Covid-19. Sticky platelet syndrome and sickle ovid-19, an abbreviation for corona cell disease are genetic disorders with a proco- virus disease 2019, is caused by se- agulant nature of the disease, while in Glanz- vere acute respiratory syndrome co- mann syndrome there is an enhanced bleeding rona virus 2 (SARS-CoV-2). Covid-19 tendency, with the pathological defect leading to pandemic, which originated in altered platelet aggregation and delayed clot for- CWuhan, China, in 2019, has led to a severe so- mation. cioeconomic and health devastation worldwide. Sticky platelet syndrome is an autosomal The severity of Covid-19 manifestation across dominant disease and a rare clinical condition communities has been countered with a dedi- with unknown etiology; it is characterized by hy- cated management approach, including social peraggregation of the platelets, with an increased isolation, quarantine management of active ca- risk of arterial and venous thrombosis and throm- ses and contacts, symptomatic medical manage- boembolism. Sticky platelet syndrome has been ment along with Covid-19 vaccination. For ful- the causative factor in nearly 21% of all arterial filling this obligation towards effective public thrombosis and 13% of venous thrombosis, with health services, every country requires adequate both conditions being of unknown origin (3). manpower of doctors and nurses, medicines, Sickle cell disease is due to mutation of beta oxygen cylinders and ventilators. This results in globin chain of hemoglobin in which glutamate an increasing global health burden, which is also is replaced by valine at the sixth amino acid posi- heavily affecting national economies, especially tion of the beta chain. According to epidemio- those of developing and underdeveloped coun- logical studies, around 20–25 million individuals tries. worldwide have sickle cell disease, and about The signs and symptoms associated with 50–80% of infants born with this disease in Afri- Covid-19 infection include fever, dry cough, sore ca die before the age of five years (4). Patients throat, body ache, headache, myalgia, fatigue, with sickle cell disease present with complains of shortness of breath, diarrhea, and sometimes recurrent chest infection, acute chest syndrome, loss of smell, taste or appetite. Severe Covid-19 painful vaso-occlusive crisis, stroke, deep vein infection is associated with dyspnea along with thrombosis and pulmonary embolism (4). hypoxemia, acute respiratory distress syndrome, Glanzmann syndrome is a very rare autoso- pneumonia, thrombosis, thromboembolism, co- mal recessive genetic disorder in which the agulopathy, arrhythmias and shock (1). Although platelets contain defective or low levels of glyco- the pandemic has been lasting for more than protein IIb/IIIa: GPIIb-IIIa. These glycoproteins 18 months, mortality rates are still rising on a are receptors for fibrinogen. Due to this, fibrino- daily basis worldwide. Many countries are facing gen bridging between platelets is not possible. a second wave of the pandemic as a result of the This will lead to a severe bleeding tendency and rapid circulation of mutant strains of SARS-CoV-2 defective haemostatic plug formation. Glanz- across continents. World Health Organization mann thrombasthenia is prevalent in about dashboard reported 146,054,107 confirmed 1:1,000,000 of the general population world- cases and 3,092,410 deaths on the 25th of April wide. But recent hospital-based research studies 2021 (2). and reports have pointed out to an increasing Increased arterial and venous thrombosis is number of cases with unexplained thrombosis, one of the most critical manifestations in criti- pulmonary embolism as well as severe proximal cally ill Covid-19 patients. Considering the deep vein thrombosis in a Glanzmann thrombas- thrombotic episodes of Covid-19, we decided to thenia variant (5). The causes of thrombosis in review the literature in order to find out which Covid-19, sticky platelet syndrome, sickle cell was the coagulant status in genetically associated disease and Glanzmann syndrome are showed diseases such as sticky platelet syndrome, sickle in Figure 1. cell disease and Glanzmann syndrome. We These facts gave us an impetus to search the planned to review various published studies with literature for understanding the clinical perspec- the aim to find whether the coagulant profiles in tive of higher risk of thrombosis and thromboem- these conditions altered the thrombotic manifes- bolism in patients with sticky platelet syndrome, Maedica A Journal of Clinical Medicine, Volume 16, No. 2, 2021 269 STICKY PLATELET SYNDROME, SICKLE CELL DISEASE AND GLANZMANN SYNDROME, AND SEVERE THROMBOSIS WITH COVID-19 FIGURE 1. Causes of thrombosis in Covid-19, sticky platelet syndrome, sickle cell disease and Glanzmann syndrome sickle cell disease and Glanzmann syndrome and DISCUSSIONS whether these conditions may promulgate se- vere thrombosis if these patients get infected ovid-19 and thrombosis with Covid-19. CThere has been a high risk of venous throm- boembolism in patients with Covid-19, whose MATERIAL AND METHODS infection with SARS-CoV-2 was found to cause a hypercoagulable state. Research carried out on e planned to have a thorough search of patients with Covid-19 hospitalized in intensive Wthe literature of all recently published ar- care units revealed that 31% to 79% of the de- ticles from various data bases such as Medline, veloped thrombosis (6). There is a cytokine storm PubMed and World Health Organization web- with increased release of pro-inflammatory sub- site. Sticky platelet syndrome and Glanzmann stances and interleukins, mainly interleukin 6, syndrome have not been very commonly dis- which indicates a progressive inflammatory re- cussed by researchers. Therefore, we decided to sponse and a hypercoagulable state of blood analyze various research and review papers pu- (7, 8). These pro-inflammatory cytokines impair blished in English, describing pathological mani- the natural anti-coagulation pathway and ob- festations and management of sticky platelet syn- scures the fibrinolysis process, leading to pro- drome, sickle cell disease and Glanzmann thrombotic changes in circulation and causing syndrome, which were conducted in the last arterial and venous thrombosis. The impairment three decades, using the above-mentioned key of prothrombotic changes is due
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