Sickle Cell Anemia Presenting with Vaso-Occlusive Pain: Should We Screen for COVID-19?

Abstract

Despite the widespread of coronavirus disease-19 (CO­VID-19) infection around the world, there are very scarce reported literature about the care of patients with a known diagnosis of hemoglobin disorders such as sickle cell disease (SCD) or thalassemia and confirmed COVID-19 infection. Thalassemia International Federation issued a position statement to include patients with thalassemia and SCD among the high-risk groups of patients. Here, we present an interesting case of a 42-year-old patient know to have SCD presenting with Vaso-occlusive (VOC) pain episode in the absence of COVID-19 signs and symptoms, who tested positive for COVID-19 infection and had a smooth recovery. This case highlights the importance of screening SCD patients presenting with VOC-related events even in the absence of COVID-19 signs and symptoms.

The Cause–Effect Dilemma of Hematologic Changes in COVID-19: One Year after the Start of the Pandemic

“…In hemoglobinopathies patients, such as sickle cell disease and thalassemia, infection is considered as one of the leading causes of mortality [ 18 , 19 ]. The reason for the higher susceptibility is the comorbidities, including ineffective erythropoiesis, chronic hemolytic anemia, iron overload, and hypercoagulability, which make them vulnerable to complications of COVID-19 infection [ 19 ].…”

“…The reason for the higher susceptibility is the comorbidities, including ineffective erythropoiesis, chronic hemolytic anemia, iron overload, and hypercoagulability, which make them vulnerable to complications of COVID-19 infection [ 19 ]. Although splenectomy (or auto-splenectomy in the case of SCD) is not known to increase the risk of severe viral infections, splenectomized patients may be at risk of severe secondary bacterial infections when infected by COVID-19 [ 18 ]. Although the experience from the 2009 H1N1 influenza pandemic has presented the H1N1 influenza virus as a trigger for acute chest syndrome (ACS) and the need for intensive-care support [ 18 , 20 ], most of the COVID-19 chronic hemoglobinopathy patients have a milder form.…”

“…Although splenectomy (or auto-splenectomy in the case of SCD) is not known to increase the risk of severe viral infections, splenectomized patients may be at risk of severe secondary bacterial infections when infected by COVID-19 [ 18 ]. Although the experience from the 2009 H1N1 influenza pandemic has presented the H1N1 influenza virus as a trigger for acute chest syndrome (ACS) and the need for intensive-care support [ 18 , 20 ], most of the COVID-19 chronic hemoglobinopathy patients have a milder form. Only in a minor percentage of patients can the more severe forms of COVID-19 pneumonia be attributed to the states of hypoxia and VQ mismatch, which sometimes complicate the normal disease course [ 18 , 21 ].…”

“…Although the experience from the 2009 H1N1 influenza pandemic has presented the H1N1 influenza virus as a trigger for acute chest syndrome (ACS) and the need for intensive-care support [ 18 , 20 ], most of the COVID-19 chronic hemoglobinopathy patients have a milder form. Only in a minor percentage of patients can the more severe forms of COVID-19 pneumonia be attributed to the states of hypoxia and VQ mismatch, which sometimes complicate the normal disease course [ 18 , 21 ]. Another consideration is the concurrent mismatch between blood demand and supply in healthcare services during the pandemic, which led to the prioritization of the needs of chronic hemolytic anemia patients [ 21 ].…”