Case of acquired or pseudo-Pelger-Huet anomaly

Abstract

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 with certain infections and drugs. We report a case of a 67-year-old Caucasian gentleman with past medical history of rheumatoid arthritis, type II diabetes mellitus and hypothyroidism, who presented with 1 day history of fever (101°F) and night sweats. Medications include ibuprofen, methotrexate, hydroxychloroquine and levothyroxine. Patient denied any other symptoms. His work-up showed normal WBC count (8.6) and increase in bands (24%). The patient was admitted for further evaluation. During the next 2 days, the patient did not have any fever or any new symptoms. Peripheral blood smear was done as part of his work-up for bandemia, showed findings suggestive of PHA. Ibuprofen was discontinued. Follow-up few weeks later showed normal blood smear. Diagnosis of PPHA was made. The presented case showed that we should think of PHA\PPHA in any case with normal total WBC count and significant shift to the lift with no apparent explanation. Looking at smears directly under the microscopes is crucial to make diagnosis.

Peripheral granular lymphocytopenia and dysmorphic leukocytosis as simple prognostic markers in COVID‐19

“…We observed neutrophil dysplasia, including APHA and other monolobated neutrophils, in mild‐to‐severe COVID‐19 and increased levels of toxic granulation/Döhle bodies in severe cases, similar to the previous reports 9,19 . Several infections, including tuberculosis and viral infections by HIV, EBV, and parvovirus, are known to cause bone marrow stress, resulting in hematopoietic dysfunction and the appearance of APHA 20 . Neutrophil anomalies, including toxic granule and Döhle bodies, appear in secondary bacterial or fungal infections, including sepsis 21 …”

Study of Neutrophil Nuclear Lobulations on Peripheral Blood Smears and Distinguishing the Bilobed Neutrophils From Pelger Huet Cells

“…2 Pelger-Huët Anomaly (PHA) is a rare benign autosomal-dominant anomaly with an incidence of ∼1 in 6000. 3 PHA can cause a false rise in band forms. 3 Pseudo -Pelger Huet anomaly has similar morphology but different aetiology like Myelodysplastic syndrome, infections, drugs etc.…”

“…3 PHA can cause a false rise in band forms. 3 Pseudo -Pelger Huet anomaly has similar morphology but different aetiology like Myelodysplastic syndrome, infections, drugs etc. 3 The cause is mutations of the Lamin B receptor (LBR) gene, 4,5 which is important for maintaining nuclear structure.…”

Inherited Disorder: Pelger-Huët Anomaly

“…9 In humans, it is found rather rarely in a heterozygous state, according to Shetty et al 10 in about one of every 5000 subjects. An incidence of one in 6000 is reported by Ayan et al 11 Presumed homozygous individuals have ovoid neutrophil nuclei, as well as varying degrees of developmental delay, epilepsy and skeletal abnormalities. 12 The human neutrophil has a lobulated nucleus containing condensed chromatin.…”

“…PHA must be distinguished from acquired or pseudo-Pelger-Huët anomaly (PPHA), which has although very similar morphologic characteristics, but has been related with pathologic conditions such as myelodysplastic syndrome, myeloproliferative disease (classically chronic myeloid leukemia, CML; acute myeloid leukemia) or myelofibrosis, as well as with certain infections (human immunodeficiency virus (HIV), TB and mycoplasma) and drugs. 11 In these conditions, nuclear bilobation is generally asymmetric and is present in roughly 25% (clonal disorders) to 50% (drug-induced) of granulocytes. 1 An acquired neutrophil dysplasia similar to PPHA also characterized by hyposegmentation of the neutrophil nucleus and excessive chromatin clumping has been described in hematological diseases and in some clinical situations, especially under the effect of certain drugs like tacrolimus, ganciclovir, co-trimoxazole, itraconazole fludarabine, rituximab, citalopram and lorazepam.…”