When Sickle Cell Disease is out of control: what failed?

Background. Sickle Cell Disease (SCD) manifestations are mainly related to hemolytic crises (HC) and vasoocclusive (VO) phenomena with ischemia and tissue damage. Blood transfusion (simple or exchange) has a fundamental role in treating anemia and VO events and in preventing chronic complications. Case report. A 36 years old african woman with SCD entered the emergency department with severe HC (Hemoglobin 3.4 g/dL), acute chest syndrome, limb acute VO pain and urinary infection. She was managed with fluids, antibiotics and transfused with 4 erytrocyte units. She had previous medical history of irregular transfusion support and non complicance to hidroxyurea therapy, despite many VO episodes. On Day 7, due to a prothrombotic state (Figure 1) anticoagulation was started. Because of persistent fever and a COVID-19 outbreak in hematology ward, SARS-CoV-2 infection was diagnosed. She was treated with non-invase ventilation, dexametasone and simple transfusion. On Day 21 she developed a Posterior Reversible Encephalopaty Syndrome, being transferred to Intensive Care Unit and managed with labetalol perfusion and phenytoin. On Day 55 and 104 she needed surgical treatment for osteomyelitis (Figure 1). On Day 88 she was diagnosed with acute pericarditis after new onset of fever. She was discharged on Day 138. Along the hospitalization, she received 23 simple blood transfusion in the context of HC and 5 as part of preoperative management. She presented previous anti-Kell alloimmunization and developed anti-E and anti-Chido/ Rogers. All but 1 unit selected were ABO/Rh/Kell, Fya and S compatible. She had 2 HbS quantifications (Figure 1). Conclusion. Therapy non compliance is associated with serious SCD complications with major impact in patients' prognosis. Delay in diagnosis and institution of proper treatment did occur and could be explained by overlapping symptoms and complex medical issues presented. We argue that, at some points, exchange transfusion could have been the right choice of care preventing some SCD complications. Long term management is also a complex issue: starting a prophylactic regular transfusion program would be challenging considering patient's alloimmunization status and history of bad compliance. Motor and functional rehabilitation plan is ongoing.