SESSION TITLE: Critical Care Student/Resident Case Report Posters I
SESSION TYPE: Student/Resident Case Report Poster
PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM
INTRODUCTION: Pelger-Huet anomaly (PHA) is a rare benign autosomal dominant anomaly with an incidence of 1 in 6000 births. PHA is characterized by morphologically abnormal neutrophils that look like band cells without any obvious neutrophilia.1
CASE PRESENTATION: A 67 year old gentleman presented with a 1-day history of fever and night sweats. The past medical history was significant for rheumatoid arthritis and diabetes mellitus type 2. Patient’s home medications included Ibuprofen 600 mg QID, methotrexate and hydroxychoroquine. On examination, the patient had thick hands without an obvious joint swelling. Vital signs were within normal limits Laboratory tests on presentation were significant for a normal white blood cell (WBC) count with elevated band forms (24%), ESR (38), and CRP (7.3). Urinalysis and chest x-ray did not show any evidence of infection. Over the next two days the patient remained afebrile and had no new signs or symptoms. A peripheral blood smear showed normal mature chromatin condensation and granulation. 20% of the neutrophils have bi-lobed nuclei with a thin filament connecting the lobes. The remaining 80% of the neutrophils showed bi-lobed nuclei without a thin filament that made them look like band cells. These findings were consistent with benign heterozygous PHA. Ibuprofen was discontinued as it was deemed as the likely culprit causative agent for acquired PHA. Follow-up visit in the clinic subsequently demonstrated resolution of the anomaly and normal neutrophils.
DISCUSSION: Normally, neutrophils have nuclei with 3-4 segments in 70-75%, 2 segments in 15-20%, more than 4 nuclei in 5 % and nuclei without any segmentation in around 3-5% of the population.2 The hallmark of PHA is hyposegmentation of the neutrophil’s nuclei with a lower nuclear/cytoplasmic ratio and abundant chromatin clumping that gives the appearance of “pince-nez” or “dumbbell”. However, overall cell size, appearance of cytoplasm and staining quality of granules are similar to normal mature neutrophils.1 Nevertheless, cells with PHA have a normal life span without any effect on their cytotoxic functions.3
CONCLUSIONS: PHA should be suspected in patients with normal WBC count and a significant shift to the left with no apparent explanation. Although it is a benign condition, it may cause the patient to be exposed to unnecessary, expensive and inconvenient laboratory tests or imaging studies. Physicians are encouraged to obtain a peripheral smear in the appropriate setting to rule out this relatively uncommon anomaly.
Reference #1: 1. Best S, Salvati F, Kallo J, et al. Lamin B-receptor mutations in pelger-huet anomaly. Br J Haematol. 2003;123(3):542-544.
Reference #2: 2. KLEIN A, HUSSAR AE, BORNSTEIN S. Pelger-huet anomaly of the leukocytes. N Engl J Med. 1955;253(24):1057-1062.
Reference #3: 3. Borovik L, Modaff P, Waterham HR, Krentz AD, Pauli RM. Pelger-huet anomaly and a mild skeletal phenotype secondary to mutations in LBR. Am J Med Genet A. 2013;161A(8):2066-2073.
DISCLOSURE: The following authors have nothing to disclose: Abd almonem Abdelrahman, Gaurav Goyal, Osama Elsallabi, Hamza Tantoush, Mahmoud Abu Hazeem
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