Impact of covid-19 on sickle cell disease in lebanon;Mild disease and one case of deep vein thrombosis
Authors: N. El Hariri, G. Siblany, R. H. Wakim, F. Haddad, G. Dbaibo, S. Khafaja, M. R. Abboud
Journal: HemaSphere (2021)

Background: Novel coronavirus SARS-CoV-2, COVID-19, infection was first reported in December 2019, and then spread dramatically around the world. Sickle cell disease is caused by a mutation in the beta globin gene resulting in the production of sickled hemoglobin that alters its shape upon deoxygenation to form an elongated rigid sickled erythrocyte that leads to vaso-occlusion. To date, there have been limited published data on how COVID-19 may affect morbidity and mortality in SCD patients. A review of the literature showed that SARS-CoV-2 infection should be considered to be an important triggering factor of sickle cell crisis. Most investigators report mild disease in children and moderate disease in many SCD adults. Aims: The aim of this report is to describe the clinical features of 11 confirmed cases of COVID-19 in patients with SCD in Lebanon. Methods: 11 SCD patients, were infected with COVID-19. Data concerning the age, gender, weight, and genotype were collected. In addition to medications received and chronic transfusion. Information about the course of COVID-19 is reported regarding: complications, type and duration of symptoms as well as therapy. Results: In this series median age was 22.2 years (range 14-40 years), only 2 are females and 8 patients had HbSS, 2 HbS beta + thalassemia, and 1 HbSC. 3 patients weighed >70kg the others were all below 70 kg. Ten patients were on long term hydroxyurea (HU) and in one it was initiated for only 1 week before infection. 1 patient was receiving Voxelotor and 1 Crizanlizumab in addition to HU. None were on chronic transfusion. Two patients had previously been started on Aspirin. One was on acencoumarolum, for a previous pulmonary embolism. One patient had asthma and was a smoker. Nine patients had moderate to severe symptoms and 2 had mild symptoms. 2 patients underwent PRBC transfusion for pain and anemia and one had erythrocytopherisis to control severe pain. Three patients required oxygen but none were admitted to a critical care unit. The mean number of days with symptoms was 14.1 days. Of the 11 patients 7 experienced pain crisis. None developed acute chest syndrome. 3 patients received corticosteroids and 5 patients received low molecular weight heparin (LMWH) 2 received therapeutic dose and 3 prophylactic dose. A 16 years old male with HbSS genotype developed multi system inflammatory syndrome and was admitted to regular floor for 26 days. He had prolonged high grade fever, epigastric pain, sore throat in addition to maculopapular rash. He had elevated D-Dimers >3000 ng/ml. He received PRBC transfusion, IVIG, corticosteroids, LMWH and antibiotics. Another 18 year old male with HbSS, developed extensive right lower limb deep vein thrombosis (DVT) 20 days after recovering from very mild COVID-19 infection. This patient had been on aspirin for 6 years after recovering from a catheter provoked DVT. Summary/Conclusion: This is one of the first reports on COVID-19 in patients who are not of African descent. In our patients COVID-19 was manifested mainly as fever and pain crises. Most patients required transfusions. Overall the symptoms were mild or moderate, similar to what was reported by others. Of concern were the development of multi system inflammatory syndrome in one patient and the development of delayed extensive DVT in another.