ISSN 2398-2950      

Pelger-Huet anomaly

ffelis

Introduction

  • Extremely rare (especially homozygous form).
  • Cause: congenital hereditary autosomal dominant disorder of leukocyte development.
  • Signs: usually benign in heterozygous form but can be lethal in homozygous form.
  • Diagnosis: often incidental finding of apparent continual left shift despite animal appearing clinically normal, ie hyposegmentation of granulocytes.
  • Treatment: none, supportive care only if evidence of infection.
  • Prognosis: survival times not reported but heterozygous animals probably can live relatively normal lives.

Pathogenesis

Etiology

  • Believed to be inherited - autosomal dominant fashion.

Heterozygous form

  • Blood granulocytes indented band-shaped bilobed nuclei with coarse mature chromatin pattern.

Homozygous form

  • Granulocytes and monocytes have round to oval nuclei with very coarse 'chunky' chromatin.
  • Usually lethal in utero in embryonic form.
  • Has been reported in association with chondrodysplasia Chondrodysplasia.

Pathophysiology

  • Benign condition characterized by hyposegmentation of granulocyte (neutrophil, eosinophil, basophil) nuclei.
  • Monocyte and megakaryocyte nuclei are also affected.
  • Stem cell defect in nuclear segmentation process   →   nuclei remain round, oval or bean shaped.
  • Reduced functionality of white cells may   →   predisposition to chronic low-grade infection but leukocyte function is the same in patients with Pelger-Huet anomaly as in normal patients in heterozygous form - no significant differences have been found in neutrophil adherence, random movement, chemotaxis, phagocytosis, or bacterial killing?
  • Acquired non-hereditary Pelger-Huet cells may be seen in other disease states, eg chronic bacterial or viral infections (eg FeLV, FIV infection), some drugs, neoplasia, myelodysplastic disease of the bone marrow.

Timecourse

  • Weeks to months.

Diagnosis

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Treatment

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Prevention

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Outcomes

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Further Reading

Publications

Refereed papers

  • Recent references from PubMed and VetMedResource.
  • Deshuillers P, Raskin R, Messick J (2014) Pelger-Huët anomaly in a cat. Vet Clin Pathol 43 (3), 337-341 PubMed.
  • Latimer K S, Rowland G N & Mahaffey M B (1988) Homozygous Pelger-Huët anomaly and chondrodysplasia in a stillborn kitten. Vet Pathol 25 (4), 325-328 PubMed.
  • Guildford W G (1987) Primary immunodeficiency diseases of dogs and cats. Comp Cont Educ Prac Vet (6), 641-50 AGRIS FAO.
  • Latimer K S, Rakich P M & Thompson D F (1985) Pelger-Huë​t anomaly in cats. Vet Pathol 22 (4), 370-374 PubMed.
  • Weber S E, Evans D A & Feldman B F (1981) Pelger-Huet anomaly of granulocytic leukocytes in two feline littermates. Feline Practice 11 (1), 44-47 VetMedResource.

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