Acquired Pelger–Huët: What does it really mean?

Abstract

Pelger-Huët anomaly (PHA) is a benign inherited condition characterized by hyposegmentation of the neutrophil's nucleus and excessive chromatin clumping. An acquired neutrophil dysplasia similar to PHA has been described in hematological diseases and in some clinical conditions. It has been known as acquired or pseudo PHA. Although some hypotheses have been proposed to explain this phenomenon, the mechanism of nuclear change is still unclear. Only the laboratory and clinical data combined will yield a better understanding on the need for follow-up and management of patients in the appropriate cases. In addition, a possible cause of pseudo PHA must always be investigated to add insights to the full understanding of this abnormality. Whether this neutrophil phenomenon has clinical implications remains to be elucidated. It is clear that only a small number of patients under drugs (immunosuppressive and others) may present these neutrophil abnormalities. Most of them do not show this phenomenon and we are unable to explain the different responses in drug users. Whether these patients display a predisposition for developing bone marrow or other diseases in the future, it is a very intriguing matter and only a follow-up will solve this question.

Homozygous Pelger-Huet anomaly in three different crossbred rabbits: a case report

“…PHA occurs in congenital and acquired forms. The acquired form is caused by drug administration or infectious diseases (Dusse et al 2010), but this was ruled out due to the absence of clinically ill rabbits and because no treatments were applied in any of cases. On the other hand, as mentioned previously, the congenital form of PHA is caused by mutations in the gene encoding the lamin B receptor which affect the structure of this protein (Hoffmann et al 2002;Best et al 2003).…”

A case report on reversible Pelger–Huët anomaly depending on serum free fraction of valproic acid

“…This disorder can also be acquired following various forms of drug therapy, including immunosuppressive therapy, colony stimulating factor, interleukin-2, nomifensine, ibuprofen, sulfonamide and d-penicillamine [4]. Only two reports have described the occurrence of VPA-induced Pelger-Huët anomalies [5,6].…”

“…Pelger-Huët anomalies occur as a dominantly inherited disorder of segmentation of polymorphonuclear leukocytes [4]. This disorder can also be acquired following various forms of drug therapy, including immunosuppressive therapy, colony stimulating factor, interleukin-2, nomifensine, ibuprofen, sulfonamide and d-penicillamine [4].…”

Pelger–Huët Anomaly in a cat

“…Furthermore, there should be no evidence of concurrent disease or treatment that might be linked to the anomalous changes. 2,3 Pseudo Pelger-Hu€ et anomaly is an acquired dysplasia of granulocytes characterized by nuclear hyposegmentation; these cells sometimes also have clumped chromatin 5,6. The main causes identified in human and veterinary medicine for pseudo Pelger-Hu€ et anomaly include infectious agents (eg, FeLV or FIV infection), severe inflammation, myeloid neoplasm (eg, myelodysplastic syndrome [MDS]), and drug toxicity 2,3,[7][8][9][10].…”