Inherited Disorder: Pelger-Huët Anomaly

Inherited Disorder: Pelger-Huët Anomaly

Submit Manuscript | http://medcraveonline.com Abbreviations: AML: Acute Myeloid Leukemia; CBC: Complete Blood Count; CML: Chronic Myeloid Leukemia; HIV: Human Immunodeficiency Virus; IFN-g: Interferon Gamma; INM: Inner Nuclear Membrane; LBR: Lamin B Receptor; MDS: Myelodysplastic Syndrome; NE: Nuclear Envelope; ONM: Outer Nuclear Membrane; PHA: Pelger-Huët Anomaly; PHA: PseudoPelger-Huët Anomal...

Understanding and recognizing the Pelger-Huët anomaly.

The Pelger-Huët anomaly (PHA) is a recognized morphologic variant affecting all granulocytes but is most evident in polymorphonuclear neutrophils (PMNs). PHA is caused by a decreased amount of the lamin B receptor (LBR). Recognition of PHA morphologic features serves as a marker for mutations in the LBR gene. This review summarizes the history of PHA and the current knowledge of the functions o...

The Pelger-Huët familial anomaly of leukocytes.

Homozygous form of the Pelger-Huët anomaly.

Pseudo-Pelger-Huët anomaly induced by medications: a clinicopathologic study in comparison with myelodysplastic syndrome-related pseudo-Pelger-Huët anomaly.

Pseudo-Pelger-Huët anomaly (PPHA) has been documented in association with transplant medications and other drugs. This iatrogenic neutrophilic dysplasia is reversible with cessation or adjustment of medications but is frequently confused with myelodysplastic syndrome (MDS) based on the conventional concept that PPHA is a marker for dysplasia. We investigated the clinicopathologic features in ia...