Pseudo Chediak-Higashi Granules
Pseudo Chediak-Higashi Granules:
This extremely impressive picture showing Pseudo Chediak-Higashi granules appear as large irregular cytoplasmic inclusions in this leukemia cell (arrow).
Pseudo Chediak-Higashi Anomaly:
PCH anomaly was first described in 1964 by Didisheim et al. Later, VanSlyck and Rebuck reported similar granules in the leukemic cells of 2 patients with AML-M4, and used the term, pseudo Chediak-Higashi anomaly of acute leukemia, because of the resemblance of the granules to those seen in patients with inherited Chediak-Higashi syndrome. 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. Most reported cases of PCH anomaly in leukemia patients are in adults; there are only a few reported childhood cases.
Ultrastructural studies of PCH anomaly have shown that the granules are strongly MPO positive, with variable positivity for PAS, Sudan black, and high-iron diamine, and without an obviously crystalline structure. Electron microscopic studies suggest that these granules are formed by the fusion of azurophilic granules. Some studies propose that these granules should be considered a morphological variant of Auer bodies; however, the pathophysiology of PCH anomaly remains unknown. The clinical significance of this abnormality has yet to be been determined, because PCH anomaly is not always associated with disseminated intravascular coagulation or any other characteristic clinical picture. Additional research is required to establish the therapeutic and prognostic relevance of PCH anomaly.(Ref:www.ncbi.nlm.nih.gov)
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This extremely impressive picture showing Pseudo Chediak-Higashi granules appear as large irregular cytoplasmic inclusions in this leukemia cell (arrow).
Pseudo Chediak-Higashi Anomaly:
PCH anomaly was first described in 1964 by Didisheim et al. Later, VanSlyck and Rebuck reported similar granules in the leukemic cells of 2 patients with AML-M4, and used the term, pseudo Chediak-Higashi anomaly of acute leukemia, because of the resemblance of the granules to those seen in patients with inherited Chediak-Higashi syndrome. 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. Most reported cases of PCH anomaly in leukemia patients are in adults; there are only a few reported childhood cases.
Ultrastructural studies of PCH anomaly have shown that the granules are strongly MPO positive, with variable positivity for PAS, Sudan black, and high-iron diamine, and without an obviously crystalline structure. Electron microscopic studies suggest that these granules are formed by the fusion of azurophilic granules. Some studies propose that these granules should be considered a morphological variant of Auer bodies; however, the pathophysiology of PCH anomaly remains unknown. The clinical significance of this abnormality has yet to be been determined, because PCH anomaly is not always associated with disseminated intravascular coagulation or any other characteristic clinical picture. Additional research is required to establish the therapeutic and prognostic relevance of PCH anomaly.(Ref:www.ncbi.nlm.nih.gov)
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