Understanding and recognizing the Pelger-Huët anomaly.
نویسندگان
چکیده
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 of the LBR. Guidance is given for distinguishing PHA from other hematologic disorders in which granulocytes may show similar changes. Recognition of PHA in the laboratory should prompt communication to the patient's physician about the possible clinical significance of this finding and the recommended screening for the anomaly in other family members by CBC and review of a peripheral blood smear.
منابع مشابه
Microsoft Word - AHA127BF
Anomalous cells of the Pelger-Huët type appear mainly in diseases affecting granulopoiesis such as myeloproliferative disorders, aplastic anemia and agranulocytosis [1]. The association of this anomaly with disorders of the lymphatic system is rare and had been described only in 1 patient with chronic lymphocytic leukemia [2]. Recently we have encountered another patient with chronic lymphocyti...
متن کاملInherited 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...
متن کاملCase of acquired or pseudo-Pelger-Huët anomaly
Pelger-Huët anomaly (PHA) is a rare benign autosomal-dominant anomaly with an incidence of ∼1 in 6000. It does not cause neutrophilia, but it can cause a false increase in band forms. It should be differentiated from acquired or pseudo-Pelger-Huët anomaly (PPHA), which has similar morphology, however; it is associated with different pathological states like Myelodysplastic syndrome, as well as ...
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ورودعنوان ژورنال:
- American journal of clinical pathology
دوره 137 3 شماره
صفحات -
تاریخ انتشار 2012