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Rapidly Progressive Acute Chest Syndrome (ACS) in a Patient with Sickle Cell Disease

Description

Chaturvedi et al identified a distinct phenotype of the acute chest syndrome associated with sickle cell disease. This involves rapid progression with organ failure and increased mortality. The authors are from Vanderbilt University, University of Amsterdam, Mount Sinai Hospital, and Rodeghier Consultants (Chicago).


Clinical and laboratory features:

(1) The patient has sickle cell disease.

(2) The patient has the acute chest syndrome.

(3) The patient develops dysfunction or failure in one or more organs:

(3a) acute renal failure

(3b) hepatic dysfunction

(3c) altered mental status

(3d) other

(4) The patient often has thrombocytopenia on admission or there is a rapid decline.

(5) There is increased risk of mortality.

 

Other diagnoses such as sepsis should be excluded.

 

Survival should be improved by early diagnosis and aggressive management. A delay in diagnosis or inappropriate therapy may increase the risk of mortality.


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