Pseudo-Chédiak–Higashi Anomaly

Abstract

Pseudo-Chédiak-Higashi (PCH) anomaly was seen in a patient with a myelodysplastic syndrome evolving from pancytopenia with normocellular marrow to a myeloblastic leukemia over a period of 72 months. This case is reported.

Multiple Pseudo-Chediak-Higashi Inclusions Associated with MYC Deletion in a Patient with Acute Myeloid Leukemia

“…The pseudo-Chédiak-Higashi (PCH) anomaly is a large pink or purple cytoplasmic granule that has only been rarely described in acute myeloid leukemia (AML) [1,2,3,4,5,6,7,8,9]. These giant granules are azurophilic and myeloperoxidase positive.…”

Pseudo Chediak-Higashi Anomaly

“…PCH anomaly is characterized by the presence of large cytoplasmic eosinophilic granules in leukemic blast cells, promyelocytes, and myelocytes. It is most often observed in patients with AML subtypes M2, M3, M4, and M5, but it is also associated with chronic myeloid leukemia, myelodysplastic syndrome, and mixed-lineage leukemias [1,2,3,4,5,6,7]. Most reported cases of PCH anomaly in leukemia patients are in adults; there are only a few reported childhood cases.…”

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

“…Similar large pink or purple cytoplasmic granules, defined as pseudo-Chédiak-Higashi (PCH) anomaly, have been described in rare cases of acute myeloid leukemia (AML). [1][2][3][4][5] These giant granules are azurophilic and myeloperoxidase-positive. Electron microscopy demonstrated small vesicles in the cytoplasm near the giant granules, 6 suggesting the granules may be formed by the fusion of primary granules and/or the fusion of small, dense vesicles.…”