eMedicine Specialties > Pediatrics: General Medicine > Hematology
Pelger-Huet Anomaly: Follow-up
Updated: Mar 25, 2009
Follow-up
Prognosis
- Individuals with Pelger-Huët anomaly (PHA) have good health, and their natural resistance to infection is unimpaired.
Miscellaneous
Medicolegal Pitfalls
- Acquired or pseudo–Pelger-Huët cells appear transiently in several disorders and may herald the development of acute or chronic myeloproliferative disorders and are among the most obvious markers of several preleukemic syndromes.
- These cells are commonly observed during the course of acute and chronic myelogenous leukemia and idiopathic myelofibrosis. They may indicate the clinical onset of these disorders months or years in advance.
- Failure to recognize the significance of pseudo–Pelger-Huët cells as early markers of impending myelodysplasia may delay diagnosis and negatively affect the patient's prognosis.
The authors and editors of eMedicine gratefully acknowledge the contributions of previous author Frank E Shafer, MD, to the original writing and development of this topic.
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References
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Cunningham JM, Patnaik MM, Hammerschmidt DE, Vercellotti GM. Historical perspective and clinical implications of the Pelger-Huet cell. Am J Hematol. Feb 2009;84(2):116-9. [Medline].
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Cohen TV, Klarmann KD, Sakchaisri K, Cooper JP, Kuhns D, Anver M, et al. The lamin B receptor under transcriptional control of C/EBPepsilon is required for morphological but not functional maturation of neutrophils. Hum Mol Genet. Oct 1 2008;17(19):2921-33. [Medline].
Oosterwijk JC, Mansour S, van Noort G, et al. Congenital abnormalities reported in Pelger-Huët homozygosity as compared to Greenberg/HEM dysplasia: highly variable expression of allelic phenotypes. J Med Genet. Dec 2003;40(12):937-41. [Medline].
Best S, Salvati F, Kallo J, et al. Lamin B-receptor mutations in Pelger-Huët anomaly. Br J Haematol. Nov 2003;123(3):542-4. [Medline].
Erice JG, Perez JM, Pericas FS. Homozygous form of the Pelger-Huët anomaly. Haematologica. Aug 1999;84(8):748. [Medline].
Jandl JH. Leukocyte anomalies. In: Blood: Textbook of Hematology. 2nd ed. Boston, Mass: Little, Brown; 1996:788.
Kalfa TA, Zimmerman SA, Goodman BK, et al. Pelger-Huët anomaly in a child with 1q42.3-44 deletion. Pediatr Blood Cancer. May 1 2006;46(5):645-8. [Medline].
Klein A, Hussar AE, Bornstein S. Pelger-Huët anomaly of the leukocytes. N Engl J Med. Dec 15 1955;253(24):1057-62. [Medline].
Matsumoto T, Harada Y, Yamaguchi K, et al. Cytogenetic and functional studies of leukocytes with Pelger-Huët anomaly. Acta Haematol. 1984;72(4):264-73. [Medline].
Further Reading
Keywords
Pelger-Huet anomaly, Pelger-Huët anomaly, Pelger-Huët nuclear anomaly, Pelger-Huet nuclear anomaly, PHA, pelgerization, LBR gene, pseudo-PHA, acquired PHA, Pelger-Huët cells, Pelger-Huet cells, 1q42, myeloid leukemia, myelodysplasia, acute lymphocytic leukemia, hydrops, ectopic calcifications, moth-eaten dysplasia syndrome, Greenberg syndrome, HEM, developmental delay, seizures, skeletal anomalies, skeletal dysplasia
Follow-up: Pelger-Huet Anomaly