Successful management of acute chest syndrome in a patient with sickle cell disease

Abstract

Although sickle cell disease (SCD) is common in endemic areas of malaria, it is one of the rare diseases in Japan. Hence, SCD and its complications are not well established in Japan. An 11-year-old girl was referred to the specialized pediatric center of our hospital. She was born in Brazil and diagnosed with SCD after birth. However, she did not have a routine checkup in Japan. Owing to influenza viral infection, she developed vaso-occlusive pain crisis (VPC) and needed hospitalization for pain management. After admission, she developed dyspnea, needing intratracheal intubation and mechanical ventilation. A chest X-ray revealed bilateral pulmonary infiltration, suggesting acute chest syndrome (ACS) complicated with SCD. Intensive care, including transfusion of red blood cells, successfully improved her condition. Reportedly, half cases of VPC develop ACS, and the mortality of ACS is very high. Hence, when managing VPC cases, the prevention of ACS, through transfusion of red blood cells or infectious control, is imperative. Thus, Japanese hematologists and pediatricians should recognize SCD and its complications owing to an anticipated increase of foreign travelers or migrants in the future.