A LJ 1 -year-old African American female patient is referred for evaluation of leukopenia. She reports she is generally in good health . but she has noted increasing shortness of breath in recent weeks. She has a history of ovarian carci noid tumor that was trea ted surgical ly. Complete blood count shows leukocyte count 2.1 00/fLL. hemoglobin 1 2 .2 g/dl. and platelet count 2 30,000/ fLl. Physical exam shows no splenomegaly or lymphadenopathy. I mages below are from CT chest and bone marrow biopsy.

As part of the evaluation, serum angiotensin-converting enzyme (ACE) level is drawn. What state-ment is true regarding the use of ACE levels in sarcoidosis?
- ACE levels are elevated in > 90% of patients with active sarcoidosis
- ACE levels greater than 1 50% of normal correlate with extrapulmonary sarcoidosis
- False positive results limit the usefulness of the assay in diagnosis and therapy
- Patients taking amiodarone should hold the medication for 48 hours prior to testing
C. While false-positive ACE elevations are not frequently encountered, the frequency is great enough to argue against the usage of ACE level to guide firm decisions in the diagnosis or treat-ment of sarcoidosis. In general, the ACE level is elevated in around 75% of patients with active disease.
No clear relationship between specific cutoffs in ACE elevation and extrapulrnonary sarcoid has been demonstrated. Amiodarone does not affect the ACE level (Ann Clin Biochem. 1989;26(Pt 1):13).
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