Congenital Pelger-Huët anomaly in a Danish/Swedish Farmdog: Case Report
© Lukaszewska et al; licensee BioMed Central Ltd. 2011
Received: 30 November 2010
Accepted: 1 March 2011
Published: 1 March 2011
A 13 year old Danish/Swedish Farmdog from Denmark was evaluated in a veterinary clinic in Warsaw, Poland for evaluation of an orthopedic problem. Radiographs revealed spondylosis and degenerative vertebral disease, which responded to treatment with anti-inflammatory medications. A predominance of hyposegmented neutrophils and eosinophils containing condensed chromatin and normal cytoplasm were identified on a routine CBC. Follow-up blood film evaluations over the course of 12 months confirmed that the hyposegmented granulocytes persisted. The majority of neutrophils contained Grade 2 nuclei (slightly indented), and the mean nuclear score varied from 1.9 to 2.3. Pelger-Huët anomaly (PHA), presumably congenital, was diagnosed based on persistent hyposegmented granulocytes in the absence of an underlying cause for acquired PHA; genetically related dogs were unavailable for testing to confirm vertical transmission. To the authors' knowledge this is the first report of PHA in a Danish/Swedish Farmdog.
Morphologic evaluation of leukocytes by microscopic examination of a blood film is an important component of the complete blood count (CBC). Even when total leukocyte numbers are within reference intervals, identification of immature hyposegmented neutrophils in increased numbers (a left shift) signifies an inflammatory leukogram. Hyposegmentation of neutrophils also occurs with Pelger-Huët anomaly (PHA). Congenital Pelger-Huët anomaly is a familial defect in granulocyte nuclear segmentation first described in humans in The Netherlands by Dutch physicians, K. Pelger and G. Huët in 1928 and 1932, respectively.[1, 2] Mutations in the lamin B receptor (LBR) have recently been identified as the cause of PHA in humans. LBR is a conserved integral membrane protein of the nuclear envelope that interacts with lamin B and heterochromatin, and has been shown to be required for the normal morphologic maturation of granulocytes.[4, 5] Granulocyte function in affected individuals appears to be normal.[4, 6–8] The hereditary form of PHA must be differentiated from pseudo-PHA, a temporary condition acquired secondary to an underlying disease or drug administration. The mechanism underlying granulocyte hypolobulation in pseudo-PHA remains to be elucidated, but reduced expression of LBR has been postulated. Here we report apparently congenital PHA in an aged Danish/Swedish Farmdog.
Results of serial CBCs from a Danish/Swedish Farmdog with Pelger-Huët anomaly.
5.4 - 8.9
0.37 - 0.55
Neutrophil nuclear segmentation grades* and mean nuclear scores (MNS) in serial CBCs from a Danish/Swedish Farmdog with Pelger-Huët anomaly.
Grade 1 (%)
Grade 2 (%)
Grade 3 (%)
Grade 4 (%)
Since first discovered, congenital PHA has been reported in a variety of animal species including dogs, cats, rabbits, mice, and horses.[11–15] The classic features recognized on blood film evaluation are hypolobulated granulocytes (neutrophils, eosinophils, and basophils) containing mature condensed nuclear chromatin. Nuclei are typically round, oval, rod or band shaped, or bilobed. Hypolobulation of monocytes and megakaryocytes has also been reported. The acquired pseudo-PHA occurs in people but reports in domestic animals are limited to cattle; one report in a dog was later found to be congenital.[16–19] Persistence of hyposegmented granulocytes over time in the absence of an underlying pathologic state may be considered de facto evidence for the congenital form of PHA, however identification of related individuals with the same morphologic abnormalities is required for definitive diagnosis.
In the current case, marked hypolobulation of neutrophils and eosinophils was documented over the course of a year in the absence of underlying disease and while the patient was not receiving medication. Unfortunately, this dog has no offspring and efforts to locate siblings in Denmark were unsuccessful; thus the diagnosis of congenital PHA is presumed rather than definitive. The Danish/Swedish Farmdog, recognized as a breed in Denmark and Sweden in 1987, is a small dog resembling a terrier but related to the pinscher family. Although PHA has been documented in many different dog breeds, there has been only one previous report from Europe.[20, 21]
To the authors' knowledge, the current case is the first in a Danish/Swedish Farmdog and the second report of canine PHA to originate from Europe. Recognizing the features of PHA on blood film evaluation, particularly in an aged dog with possible underlying disease, is important to avoid misidentification of an inflammatory left shift.
- Huet GJ: Ueber eine bisher unbekannte familiaere Anomalie der Leukocyten [About a previously unknown familial anomaly of leukocytes]. Klin Wochenschr. 1932, 11: 1264-1266. 10.1007/BF01758934. (In German)View Article
- Pelger K: Demonstratie van een paar zeldzaam voorkomende typen van bloedlichaampjes en bespreking der patienten [Demonstration of rare types of blood cells and discussion of patients]. Ned Tijdschr Geneeskd. 1928, 72: 1178-(In Deutsch)
- Hoffmann K, Dreger CK, Olins AL, Olins DE, Shultz LD, Lucke B, Karl H, Kaps R, Muller D, Vaya A: Mutations in the gene encoding the lamin B receptor produce an altered nuclear morphology in granulocytes (Pelger- Huët anomaly). Nat Genet. 2002, 31: 410-414.PubMed
- Cohen TV, Klarmann KD, Sakchaisri K, Cooper JP, Kuhns D, Anver M, Johnson PF, Williams SC, Keller JR, Stewart CL: The lamin B receptor under transcriptional control of C/EBPepsilon is required for morphological but not functional maturation of neutrophils. Hum Mol Genet. 2008, 17: 2921-2933. 10.1093/hmg/ddn191.PubMed CentralView ArticlePubMed
- Hoffmann K, Sperling K, Olins AL, Olins DE: The granulocyte nucleus and lamin B receptor: avoiding the ovoid. Chromosoma. 2007, 116: 227-235. 10.1007/s00412-007-0094-8.View ArticlePubMed
- Speeckaert MM, Verhelst C, Koch A, Speeckaert R, Lacquet F: Pelger- Huët anomaly: a critical review of the literature. Acta Haematol. 2009, 121: 202-206. 10.1159/000220333.View ArticlePubMed
- Latimer KS, Kircher IM, Lindl PA, Dawe DL, Brown J: Leukocyte function in Pelger- Huët anomaly of dogs. Journal of leukocyte biology. 1989, 45: 301-310.PubMed
- Latimer KS, Prasse KW: Neutrophilic movement of a Basenji with Pelger- Huët anomaly. American journal of veterinary research. 1982, 43: 525-527.PubMed
- Dusse LM, Moreira AM, Vieira LM, Rios DR, Morais ESRM, Carvalho MD: Acquired Pelger- Huët: What does it really mean?. Clin Chim Acta. 2010
- Best S, Salvati F, Kallo J, Garner C, Height S, Thein SL, Rees DC: Lamin B-receptor mutations in Pelger- Huët anomaly. Br J Haematol. 2003, 123: 542-544. 10.1046/j.1365-2141.2003.04621.x.View ArticlePubMed
- Bowles CA, Alsaker RD, Wolfle TL: Studies of the Pelger- Huët anomaly in foxhounds. The American journal of pathology. 1979, 96: 237-248.PubMed CentralPubMed
- Latimer KS, Rakich PM, Thompson DF: Pelger- Huët anomaly in cats. Veterinary pathology. 1985, 22: 370-374.PubMed
- Gill AF, Gaunt S, Sirninger J: Congenital Pelger- Huët anomaly in a horse. Veterinary clinical pathology/American Society for Veterinary Clinical Pathology. 2006, 35: 460-462.View ArticlePubMed
- Green MC, Shultz LD, Nedzi LA: Abnormal nuclear morphology of leukocytes in the mouse mutant ichthyosis. Transplantation. 1975, 20: 172-175. 10.1097/00007890-197508000-00014.View ArticlePubMed
- Nachtsheim H: The Pelger-anomaly in man and rabbit; a mendelian character of the nuclei of the leucocytes. J Hered. 1950, 41: 131-137.PubMed
- Latimer KS, Duncan JR, Kircher IM: Nuclear segmentation, ultrastructure, and cytochemistry of blood cells from dogs with Pelger- Huët anomaly. Journal of comparative pathology. 1987, 97: 61-72. 10.1016/0021-9975(87)90128-9.View ArticlePubMed
- Carper HA: Pseudo-Pelger neutrophils in the cow. Vet Med Small Anim Clin. 1965, 60: 997-998.PubMed
- Osburn BI, Glenn BL: Acquired Pelger Huët anomaly in cattle. Journal of the American Veterinary Medical Association. 1968, 152: 11-16.PubMed
- Shull RM, Powell D: Acquired hyposegmentation of granulocytes (pseudo-Pelger- Huët anomaly) in a dog. Cornell Vet. 1979, 69: 241-247.PubMed
- Latimer KS, Campagnoli RP, Danilenko DM: Pelger- Huët anomaly in Australian Shepherds: 87 cases (1991-1997). Comparative Haematology International. 2000, 10: 9-13. 10.1007/s005800070021.View Article
- Kiss M, Komar G: Pelger-Huët'sche Kernanomalie der Leukozyten bei einem Hunde [Pelger-Huet nuclear anomaly in leukocytes in a dog]. Berl Munch Tierarztl Wochenschr. 1967, 80: 474-476. In German with summary in EnglishPubMed
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