How Important Is Thrombocytosis for Prognosis of Mds with 3q21q26 Syndrome?

The structural changes involving the long arm of chromosome 3 at bands 3q21 and 3q26.2 in the form of inversion are named paracentric inversion inv(3)(q21q26.2) and in myeloid neoplasms have long been recognized, but are rare. The 3q21q26 syndrome usually occurs in a high-risk myelodysplastic syndrome (MDS) or the setting of acute myeloid leukemia (AML) and is most commonly reported as inv(3)(q21q26.2). Myeloid neoplasms with inv(3) are rare disorders with an incidence of 1% in MDS and AML. Thus, this report aims to show a patient with MDS and high platelets count who presented inv(3)(q21q26.2). A 72-year-old woman looked for medical attention due to fatigue and weakness. The patient reported a history of smoking for 41 years and denied any exposure to toxic agents. At physical examination, only pale was detected. A complete blood count revealed hemoglobin 7g/dL, MCV = 94 fL, leukocytes 5,600/mm3, neutrophils 2,242/mm3 and thrombocytosis with a platelet count of 514,000/mm3. Bone marrow aspirate showed dyserythropoiesis in 30% of cells and 6,5% blasts. The bone marrow cytogenetic analysis showed 46,XX,inv(3)(q21q26.2)[16]/46,XX[4]. The diagnosis of MDS with excess blasts - 1 was established according to the 2016 World Health Organization classification and International Prognostic Scoring System was very high. While waiting for beginning treatment, the patient died of respiratory failure due to COVID-19. Myelodysplastic syndrome with inv(3)(q21q26.2) is a rare aggressive disorder that occurs in less than 1% of all MDS cases and has been associated with a poor outcome: chemoresistance, high risk of leukemic transformation and short survival. Our case showed thrombocytosis with a platelet count of 514,000/mm3. The incidence of thrombocytosis in MDS has been reported in 8% of cases with platelets > 400 x109/L. The major report which evaluated thrombocytosis in MDS studied 2,042 cases, detecting high platelets count in 5% of cases (102/2,042). It appears that thrombocytosis does not adequately capture the aggressive nature of inv(3)(q21q26.2) in MDS but still plays an important role in the pathogenesis of this heterogeneous and dynamic disease. Our patient reported herein showed dyserythropoiesis in 30% of cells and 6,5% blasts, but nothing was detected regarding megakaryocytic lineage. Our patient died very soon after diagnosis due to viral infection. Thrombocytosis is an unusual clinical feature in MDS associated with inv(3)(q21q26.2) and the unfavorable prognosis of inv(3) is independent of thrombocytosis. Copyright © 2022

1-year mortality after myocardial injury in COVID-19 patients

Background: Myocardial injury is a known complication of COVID-19 and is related to a worse prognosis on admission. However, its impact on 1-year mortality is unknown. Methods: Retrospective cohort study with patients admitted to intensive care and confirmed diagnosis of COVID-19 by RT-PCR and with at least one measurement of troponin during hospitalization. The study period was from March/2020 to June/2021. Clinical characteristics and the occurrence of myocardial were assessed between deaths and survivors using the chi-square test and Student's t-test. Variables with p<0.01 in the univariate analysis were included in the Cox regression model to identify predictor variables of 1-year mortality. Results: 1037 patients were included, with a mean follow-up of 1.06±0.58 years, mean age = 59.9±16.2 years, and 62.7% men. The prevalence of myocardial injury was 42.8% and occurred 204 deaths (19.7%). In the univariate analysis, the variables associated with 1-year mortality were: myocardial injury (OR 7.5;CI95% 5.2–10.9), age >60 years (OR 5.65;CI95% 3.9–8.2), arterial hypertension (OR 2.8;CI95% 2.0–3.9), diabetes (OR 2.3;CI95% 1.6–3.1), chronic kidney failure (OR 3.9;CI95% 2.2–6.8), dementia (OR 1.8;CI95% 1.2–2.6) and mechanical ventilation (OR 50.5;CI95% 33.9–77.3). In Cox regression, the predictor variables were: myocardial injury (HR 2.4;CI95% 1.7–3.5), age >60 years (HR 2.5;CI95% 1.8–3.6), chronic kidney disease (HR 1.9;CI95% 1.2–2.9), dementia (HR 3.2;CI95% 2.1–5.0) and mechanical ventilation (HR 17.5;CI95% 12.2–25.2). Conclusion: In patients admitted to intensive care by COVID-19, the detection of myocardial injury more than doubled the risk of death in 1 year. Funding Acknowledgement: Type of funding sources: None.Figure 1

Diagnostic Challenge in a Sickle Cell Disease Patient with COVID-19

Acute chest syndrome is a life-threatening complication in sickle cell disease. Infections are frequently implied, and like other viruses, severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) may be a trigger. In addition, due to their inflammatory status, they may present a higher risk for severe coronavirus disease 2019 (COVID-19). Pneumonia and acute chest syndrome share clinical, laboratory, and radiological features and may overlap, which makes their differential diagnosis especially challenging. We describe a case of an adolescent with homozygous sickle cell disease that developed acute chest syndrome in the context of COVID-19. With it, we intend to bring awareness to the potential role of imaging in the differential diagnosis and in establishing the best approach for the patient. Chest computed tomography findings were suggestive of an alternative diagnosis to COVID-19 pneumonia and red cell transfusion, fluid management, analgesics, and antibiotics were administered with favorable outcome. © Author(s) (or their employer(s)) and Portuguese Journal of Pediatrics 2022.

The impact of the COVID-19 pandemic on antiallergics' prescription trends

Background: During the COVID-19 pandemic, the Portuguese government implemented several stringent measures and all the health care sectors were reorganized. The goal of this study was to assess the antiallergic prescription tendency in outpatient settings in Portugal, including in public primary health care (PC) and hospital care (HC) centers, and to evaluate potential changes during the COVID-19 pandemic. Method: The data on antiallergic prescription, in the form of monthly Defined Daily Doses (DDD), prescribed by physicians of the public health sector, were retrieved from the System of Information and Monitoring of the Portuguese National Health System (SIM@SNS) public-access platform, from January 2018 to October 2020. We used the student's t-test with 95% confidence level to compare antiallergics' prescription between the analyzed years. Results: Antiallergics' prescription in outpatient settings of PC/HC (Figure 1) presented a seasonal pattern: with increased peaks being observed throughout autumn and winter and decreased peaks in summer, with the exception of an usual spike occurring in May. In 2020, the monthly average prescription (PC= 2 747 409 ± 824 538;HC= 1 237 395 ± 289 133) was found to be lower than in 2018 (PC= 3 185 220 ± 605 702;HC =1 392 217 ± 159 337) and 2019 (PC= 3 327 975 ± 688 781;HC=1 489 159 ± 213 014), p>0.05 (in PC and HC when comparing with 2018 and 2019). In 2020, upon COVID-19 emergence, an overall decrease on antiallergics' prescription was observed, being particularly noticeable and statistically significant (p>0.05) on PC (p = 0,028) and HC (p = 0,037) centers after May. Conclusion: A significant decline on antiallergics' prescription was detected in 2020, mainly over the spring/summer months following COVID-19 emergence, probably due to the reduction in all non-essential health care activity, especially in HC. Another interesting outcome is the absence of the usual spike in May 2020, as this period was coincident with the home confinement and routine mask use, which may have reduced patients contact with outdoor allergens. (Table Presented).