Disease relevance of Pelger-Huet Anomaly

• The Pelger-Huet anomaly may be mistaken for an elevated band count, the macrocytic anemia appears not to be secondary to a vitamin B12 or folate deficiency, and the thrombocytopenia may be sensitive to drug dosage [1].

High impact information on Pelger-Huet Anomaly

• Mutations in the gene encoding the lamin B receptor produce an altered nuclear morphology in granulocytes (Pelger-Huët anomaly) [2].
• The fact that the healthy mother of the fetus showed hypolobulated nuclei in 60% of her granulocytes confirms that classic Pelger-Huët anomaly represents the heterozygous state of 3beta-hydroxysterol delta(14)-reductase deficiency [3].
• Reversible Pelger-Huët anomaly associated with ibuprofen therapy [4].
• Pelger-Huet anomaly and d-penicillamine [5].
• Translocation T(4;21) associated with the Pelger-Hüet anomaly in a patient with Ph chromosome-negative CML [6].

Chemical compound and disease context of Pelger-Huet Anomaly

• Acquired Pelger-Huët anomaly in association with concomitant tacrolimus and mycophenolate mofetil in a liver transplant patient: a case report and review of the literature [7].

Biological context of Pelger-Huet Anomaly

• Differentiation of human promyelocytic HL-60 cells into neutrophil-like cells was also accompanied by the down-regulation of lamins A/C but not of lamin B. Moreover, when compared with normal cells, neutrophils with the Pelger-Huët anomaly of nuclear hyposegmentation exhibited significantly lower activity of caspase-6, a lamin A/C-cleaving enzyme [8].

Anatomical context of Pelger-Huet Anomaly

• Heterozygous mutations in LBR cause Pelger-Huët anomaly, characterized by morphologically abnormal neutrophil nuclei, and homozygous mutations cause HEM/Greenberg skeletal dysplasia, characterized by developmental abnormalities and 3 beta-hydroxysterol-epsilon-14-reductase deficiency [9].

Analytical, diagnostic and therapeutic context of Pelger-Huet Anomaly

• We report a case of a 56-year-old man who developed acquired Pelger-Huët anomaly following liver transplantation while on the immunosuppressive agents tacrolimus and mycophenolate mofetil [7].

References