Acute Myeloid Leukemia With Pseudo–Chédiak-Higashi Anomaly Exhibits a Specific Immunophenotype With CD2 Expression

Abstract

Acute myeloid leukemia (AML) with pseudo-Chèdiak-Higashi (PCH) anomaly is a rare morphologic entity. We characterized 5 cases by multiparameter flow cytometry and found that in all cases, the blasts aberrantly expressed CD2, a pan-T cell-associated marker, in addition to their myeloid-associated markers. In contrast, CD2 was expressed in only 25 (17.9%) of 140 cases of newly diagnosed AML without PCH anomaly. CD2 expression correlated strongly with AML with PCH anomaly (P < .01), suggesting a link between a specific immunophenotypic marker, CD2, and AML with PCH anomaly.

Pseudo‐Chédiak–Higashi anomaly in acute myeloid leukemia

“…4 Auer rods are also not infrequently present. An association with t(8;21) has been reported in at least three patients, 3,5,6 but no other consistent cytogenetic abnormality has been recognized. This patient is exceptional in that the granules were confined to mature granulocytes.…”