P0443 - A case of severe multi-system disease in a sickle cell SC patient caused by Parvovirus B19 infection

Sickle cell disease (SCD) is an inherited hemoglobinopathy, which results in the production of abnormal hemoglobin S leading to polymerization, hemolysis, vaso-occlusive events, and end organ damage. Most common genetic variants of SCD are HbSS, HbSC and HbS beta thalassemia. HbSC disease is generally considered to be a variant of SCD, and despite sharing similar clinical complications, it is thought to be a milder disease [1]. Among infectious complications in patients with sickle cell disease is transient red cell aplasia caused by parvovirus B19. However, parvovirus B19 infection in the pediatric population can have various manifestations [2].

Here we present a 14-year-old female with HbSC disease who presented to the emergency room with complaint of abdominal pain and found to have splenic sequestration. Within 24 hours of hospitalization, she developed worsening splenic sequestration, dropped her hemoglobin (Hb) to 4.6 g/dl and developed acute chest syndrome (ACS). She was treated following the ACS protocol, received 4 units of packed red blood cells (PRBC) and subsequently underwent a single volume PRBC exchange transfusion. Considering her unusual presentation, with severe ARDS from alveolar hemorrhage requiring mechanical ventilation and multi-organ involvement, several autoimmune and infectious conditions with a cytokine storm component including COVID-19 disease, were considered. She was tested for COVID-19 three times and they were all negative. Results of viral studies revealed parvovirus B19 IgM antibodies signifying an acute infection. She fully recovered with supportive care and was discharged home.

Multisystem involvement simulating connective tissue disorders or malignancies with acute parvovirus B19 infection has been reported and is considered extremely rare [2]. To our knowledge, despite the overlap between some of the findings described in our patient and the ones reported in the literature, there were no reports of pediatric patients with SC disease presenting with splenic sequestration and ACS in the setting of a severe parvovirus B19 multisystem disease [3,4,5]. Although there are some case reports that showed clinical improvement with the use of steroids in such patients, our patient recovered with supportive care only [6].