26 M pmh sickle cell disease p/w chest pain, sob, fever, and cough. She is noted to be hypoxic, anemic and has new pulmonary infiltrates on cxr. What diagnosis must be considered?
A:
Acute chest syndrome
“Acute chest syndrome is a clinical entity marked by hypoxia, fever and pulmonary infiltrates. Many EPs are confused by this because the definition of acute chest syndrome is identical to the definition for pneumonia. There are essentially two reasons that acute chest syndrome is defined as a clinical entity separate from pneumonia: 1) it is not always caused by infection and 2) acute chest syndrome must be treated with exchange transfusion in addition to standard pneumonia therapy. Clinically, patients who respond well to standard pneumonia therapy are labeled as having pneumonia. Those who become progressively more hypoxic despite antibiotics and fluids are labeled as acute chest syndrome and will likely need exchange transfusion to save their life. In the event that exchange is not available, a simple red cell transfusion may be adequate.”
REF:
Jeffrey Glassberg MD. Sickle Cell: 10 Things Every EP Should Know about SCD
http://www.epmonthly.com/cme/current-issue/sickle-cell-10-things-every-ep-should-know-about-scd-/1/
