65 Pelger-Huet Anomaly
Michelle To and Valentin Villatoro
Images of Pelger-Huet Anomaly in various peripheral blood smears showing numerous hyposegmented neutrophils with mature clumped chromatin. From MLS Collection, University of Alberta.
Image 1: 50x oil immersion. https://doi.org/10.7939/R3DB7W572
Image 2: 50x oil immersion. https://doi.org/10.7939/R32Z13500
Image 3: 50x oil immersion. https://doi.org/10.7939/R3Z60CH59
PBS Key Features:1-4
Neutrophil nuclei appear hyposegmented – can appear as a single round nucleus (unilobed, homozygous Pelger-Huet Anomaly) or dumbbell shaped (bilobed, heterozygous Pelger-Huet Anomaly). Anomaly is differentiated from a left shift by displaying mature chromatin pattern, abundant cytoplasm (low nuclear:cytoplasmic ratio), mature granulation, and an absence of toxic changes.
Congenital Pelger-Huet: granulocytes show normal granulation, 50-90% of neutrophils are affected.
Pseudo Pelger-Huet: seen in leukocyte malignancies and Myelodysplastic Syndrome, hypogranulation and other Dy’s plastic features may be present, 10-30% of neutrophils are affected.
Clinical Significance and Cause:1,3,5
Pelger-Huet Anomaly is benign and cell function is normal. Psuedo Pelger-Huet may indicate leukocyte malignancies and myelodysplasia.
Congenital: Lamin β-receptor gene mutation.
Acquired (Pseudo-Pelger-Huet): Hematologic malignancies such as myelodysplastic syndrome (MDS), acute myeloid leukemia (AML), myeloproliferative neoplasms (MPNs). Pseudo-Pelger-Huet may also be seen during infections, and drug interactions.
Congenital Pelger-Huet: Cytopenias often absent
Pseudo-Pelger-Huet: Cytopenias often present
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