Atraumatic splenic rupture in COVID-19 era: case report and systematic literature review.

Atraumatic splenic rupture is a rare but life-threatening condition. The Coronavirus disease 2019 (COVID-19) is still a mysterious infection, often associated with spontaneous bleeding and coagulation disorders. Among them, a rare presentation is the atraumatic splenic rupture during SARS-CoV-2 infection. We reported the case of a COVID-19 patient that underwent splenectomy for a spontaneous splenic rupture without splenomegaly or any hematological disorders. Moreover, we reviewed the literature about this matter focusing on the hypothetical etiopathogenesis of this condition in COVID-19 patients. Twelve cases of atraumatic splenic rupture without splenomegaly were reported in ten papers. Coughing, respiratory tract infections and anticoagulant treatment were postulated as the main risk factors for the spontaneous splenic rupture. COVID-19 is still a not well-known disease with multiple clinical presentations. The spontaneous splenic rupture is uncommon in general population but this event should not be neglected in this particular subset of patients.

Severe refractory warm autoimmune haemolytic anaemia after the SARS-CoV-2 Pfizer-BioNTech vaccine (BNT162b2 mRNA) managed with emergency splenectomy and complement inhibition with eculizumab.

A male in his teens with a history of liver transplant for biliary atresia (aged 2 years) and autoimmune haemolytic anaemia (AIHA, aged 6 years) presented with jaundice, dark urine, fatigue and chest discomfort that began 48 hours after the first dose of SARS-CoV-2 Pfizer-BioNTech vaccine (BNT162b2 mRNA). Investigations revealed a warm AIHA picture. Over 4 weeks the patient developed life-threatening anaemia culminating in haemoglobin of 35 g/L (after transfusion), lactate dehydrogenase of 1293 units/L and bilirubin of 228 µmol/L, refractory to standard treatment with corticosteroids and rituximab. An emergency splenectomy was performed that slowed haemolysis but did not completely ameliorate it. Eculizumab, a terminal complement pathway inhibitor, was initiated to arrest intravascular haemolysis and showed a favourable response. AIHA is rare but described after the SARS-CoV-2 Pfizer-BioNTech vaccine. This case highlights the rare complication of AIHA, the use of emergency splenectomy for disease control, and the use of eculizumab.

Splenectomy and risk of COVID-19 infection, hospitalisation, and death.

OBJECTIVES: Splenectomy is a common surgical procedure, and splenectomized patients have shown to be severely more affected by certain infections than patients with a preserved splenic function. We investigated the risk of COVID-19 infection and subsequent hospitalisation and death in splenectomized patients. METHODS: We conducted a case-control study of all individuals with a microbiologically verified COVID-19 infection in Denmark through December 31, 2020. To each case, we matched three controls on age, sex, and region of residence. We examined the association between previous splenectomy and the risk of COVID-19 infection, hospitalisation, and death using a logistic regression model. RESULTS: We identified 165,623 individuals with a positive COVID-19 test and 493,300 matched controls. Mean age was 38 years. 130 and 422 splenectomies were performed in the COVID-19 positive individuals and controls, respectively. Splenectomized patients did not have a higher risk of COVID-19 infection than non-splenectomized patients (adjusted OR: 0.89; 95% CI: 0.73-1.08). Among COVID-19 positive individuals, splenectomized patients may have an increased risk of hospitalisation or death (adjusted OR for combined endpoint: 1.44; 95% CI: 0.79-2.61). CONCLUSIONS: Splenectomized patients are not at an increased risk of COVID-19 infection, but they may have a higher risk of hospitalisation or death among COVID-19 positive individuals. This may be attributed to higher comorbidity levels.

Morbidity and Mortality in Patients with Perioperative COVID-19 Infection: Prospective Cohort in General, Gastroesophagic, Hepatobiliary, and Colorectal Surgery.

BACKGROUND: Severe acute respiratory syndrome due to coronavirus 2 has rapidly spread worldwide in an unprecedented pandemic. Patients with an ongoing COVID-19 infection requiring surgery have higher risk of mortality and complications. This study describes the mortality and morbidity in patients with perioperative COVID-19 infection undergoing elective and emergency surgeries. METHODS: Prospective cohort of consecutive patients who required a general, gastroesophageal, hepatobiliary, colorectal, or emergency surgery during COVID-19 pandemic at an academic teaching hospital. The primary outcome was 30-day mortality and major complications. Secondary outcomes were specific respiratory mortality and complications. RESULTS: A total of 701 patients underwent surgery, 39 (5.6%) with a perioperative COVID-19 infection. 30-day mortality was 12.8% and 1.4% in patients with and without COVID-19 infection, respectively (p < 0.001). Major surgical complications occurred in 25.6% and 6.8% in patients with and without COVID-19 infection, respectively (p < 0.001). Respiratory complications occurred in 30.8% and 1.4% in patients with and without COVID-19 infection, respectively (p < 0.001). Mortality due to a respiratory complication was 100% and 11.1% in patients with and without COVID-19 infection, respectively (p < 0.006). CONCLUSIONS: 30-day mortality and surgical complications are higher in patients with perioperative COVID-19 infection. Indications for elective surgery need to be reserved for non-deferrable procedures in order to avoid unnecessary risks of non-urgent procedures.

Preventing infections in children and adults with asplenia.

An estimated 1 million people in the United States have functional or anatomic asplenia or hyposplenia. Infectious complications due to encapsulated organisms such as Streptococcus pneumoniae, Neisseria meningitidis, and Haemophilus influenzae can lead to fulminant sepsis and death, particularly in young children, in the period shortly after splenectomy, and in immunocompromised patients. Patients with asplenia are also at risk for less common infections due to Capnocytophaga, Babesia, and malaria. Antibiotic prophylaxis, vaccines, and patient and family education are the mainstays of prevention in these at-risk patients. Recommendations for antibiotic prophylaxis typically target high-risk periods, such as 1 to 3 years after splenectomy, children ≤5 years of age, or patients with concomitant immunocompromise. However, the risk for sepsis is lifelong, with infections occurring as late as 40 years after splenectomy. Currently available vaccines recommended for patients with asplenia include pneumococcal vaccines (13-valent pneumococcal conjugate vaccine followed by the 23-valent pneumococcal polysaccharide vaccine), meningococcal vaccines (meningococcal conjugate vaccines for serogroups A, C, Y and W-135 and serogroup B meningococcal vaccines), H. influenzae type b vaccines, and inactivated influenza vaccines. Ongoing booster doses are also recommended for pneumococcal and meningococcal vaccines to maintain protection. Despite the availability of prevention tools, adherence is often a challenge. Dedicated teams or clinics focused on patient education and monitoring have demonstrated substantial improvements in vaccine coverage rates for individuals with asplenia and reduced risk of infection. Future efforts to monitor the quality of care in patients with asplenia may be important to bridge the know-do gap in this high-risk population.

ß-Thalassemia Major and Coronavirus-19, Mortality and Morbidity: a Systematic Review Study.

ß-Thalassemia (ß-thal) is one of the most common inherited diseases all over the world. These patients are very susceptible to infection disease, and the mortality and morbidity of infected patients will increase. The object of this systematic review study was to determine mortality and morbidity of infected ß-thal patients with coronavirus disease 2019 (COVID-19). We searched PubMed, Elsevier, and Scholar Google to obtain related papers. The time of search was 21 June until 17 July 2020. All original and review articles and case reports were searched with key words: COVID 19, beta or ß-thalassemia (ß-thal), mortality and morbidity. Data were extracted after quality assessment of all articles. We obtained seven, 21 and six articles from PubMed, Scholar Google and Science Direct, respectively. Finally, seven articles were discussed in our study. The total number of enrolled patients was 34. Twenty-six patients carried transfusion-dependent ß-thal major (ß-TM). The most common symptoms were fever, cough, pain and dyspnea. Nine patients died. The result of this study has shown that the mortality and morbidity of infected ß-thal patients will escalate.

Direct and indirect effects of the SARS-CoV-2 pandemic on Gaucher Disease patients in Spain: Time to reconsider home-based therapies?

OBJECTIVE: An analysis of the SARS-CoV-2 pandemic impact in the Spanish Gaucher Disease (GD) community is presented here. PATIENTS & METHODS: The Spanish GD foundation (FEETEF) surveyed 113 GD patients from March 30 to April 27; all patients provided a verbal consent. RESULTS: 110 surveys were analyzed. The median age was 47 years old (y.o.), 31 patients were ≥ 60 y.o.; and 34% of patients reported comorbidities. 46% (51/110) of patients were treated by enzyme replacement therapy (ERT), 48 of them at hospitals; 45.1% (45/110) were on substrate reduction therapy (SRT) and 9% (10/110) receive no therapy. 25% (11/48) of ERT-hospital-based patients reported therapy interruptions, while SRT-patients did not report missing doses. No bone crises were reported. However, 50% (55/110) of patients reported being worried about their predisposition to a severe SARS-COV-2 infection and 29% (16/55) of them took anxiolytics or antidepressants for this. While 6 patients reported to have contact with an infected person, another two confirmed SARS-CoV-2 infections were reported in splenectomyzed patients, one of them (a 79-year-old diabetic) died. CONCLUSIONS: One quarter of the patients treated at hospitals reported dose interruptions. Home-based therapy may need to be considered in order to minimize the impact of the COVID-19 pandemic.