A 32 -year-old female is found on routine physical examination to have splenomegaly. She reports no acu te change in her energy levels. However. she does note bei ng more easily fatigued duri ng heavy physical activity as long as she can remember. Patien t re-ports a h istory of cholelithiasis diagnosed 1 0 years previously; however. she elected not to undergo cholecystectomy. Mild splenomegaly is noted on physical examination . Laboratory workup reveals hemoglobin 11 .2 g/dl. mean corpuscular volume (MCV) 85 fl. leu kocyte count 6.500/,LLL. and platelet count of 361 . 000/,LLL. I ndirect bil irubin level ismini mall el vated and tneoireccanriglooin--resns negative. -reriph-eraJ-blood· smear is shown below.
What would be the most sensitive and specific test to confirm the diagnosis?
- Osmotic fragility test
- Cryohemolysis test
- Eosin-5-maleimide binding test (EMA)
- Incubation of red blood cells with diepoxybutane (DEB)
- Spleen biopsy
Medicine
C. EMA testing is a flow cytometry-based test which provides a rapid diagnosis of HS with minimal volume of required red blood cells. Sensitivity and specificity are much higher compared with other available tests and are reported to be 92.7% and 99.1 %, respectively. The sensitivity and specific-ity of osmotic fragility testing is considerably lower at 68% to 81% (Haematologica. 2012;97(4):5 16, Br J Haematol. 2000;111(3):924).
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