11.4: Pelger-Huet Anomaly
- Page ID
- 38829
\( \newcommand{\vecs}[1]{\overset { \scriptstyle \rightharpoonup} {\mathbf{#1}} } \)
\( \newcommand{\vecd}[1]{\overset{-\!-\!\rightharpoonup}{\vphantom{a}\smash {#1}}} \)
\( \newcommand{\id}{\mathrm{id}}\) \( \newcommand{\Span}{\mathrm{span}}\)
( \newcommand{\kernel}{\mathrm{null}\,}\) \( \newcommand{\range}{\mathrm{range}\,}\)
\( \newcommand{\RealPart}{\mathrm{Re}}\) \( \newcommand{\ImaginaryPart}{\mathrm{Im}}\)
\( \newcommand{\Argument}{\mathrm{Arg}}\) \( \newcommand{\norm}[1]{\| #1 \|}\)
\( \newcommand{\inner}[2]{\langle #1, #2 \rangle}\)
\( \newcommand{\Span}{\mathrm{span}}\)
\( \newcommand{\id}{\mathrm{id}}\)
\( \newcommand{\Span}{\mathrm{span}}\)
\( \newcommand{\kernel}{\mathrm{null}\,}\)
\( \newcommand{\range}{\mathrm{range}\,}\)
\( \newcommand{\RealPart}{\mathrm{Re}}\)
\( \newcommand{\ImaginaryPart}{\mathrm{Im}}\)
\( \newcommand{\Argument}{\mathrm{Arg}}\)
\( \newcommand{\norm}[1]{\| #1 \|}\)
\( \newcommand{\inner}[2]{\langle #1, #2 \rangle}\)
\( \newcommand{\Span}{\mathrm{span}}\) \( \newcommand{\AA}{\unicode[.8,0]{x212B}}\)
\( \newcommand{\vectorA}[1]{\vec{#1}} % arrow\)
\( \newcommand{\vectorAt}[1]{\vec{\text{#1}}} % arrow\)
\( \newcommand{\vectorB}[1]{\overset { \scriptstyle \rightharpoonup} {\mathbf{#1}} } \)
\( \newcommand{\vectorC}[1]{\textbf{#1}} \)
\( \newcommand{\vectorD}[1]{\overrightarrow{#1}} \)
\( \newcommand{\vectorDt}[1]{\overrightarrow{\text{#1}}} \)
\( \newcommand{\vectE}[1]{\overset{-\!-\!\rightharpoonup}{\vphantom{a}\smash{\mathbf {#1}}}} \)
\( \newcommand{\vecs}[1]{\overset { \scriptstyle \rightharpoonup} {\mathbf{#1}} } \)
\( \newcommand{\vecd}[1]{\overset{-\!-\!\rightharpoonup}{\vphantom{a}\smash {#1}}} \)
https://pressbooks.library.ualberta.ca/mlsci/?p=643
Images of Pelger-Huet Anomaly in various peripheral blood smears showing numerous hyposegmented neutrophils with mature clumped chromatin. From MLS Collection, University of Alberta.
Image 1: 50x oil immersion. https://doi.org/10.7939/R3DB7W572
Image 2: 50x oil immersion. https://doi.org/10.7939/R32Z13500
Image 3: 50x oil immersion. https://doi.org/10.7939/R3Z60CH59
PBS Key Features:1-4
Neutrophil nuclei appear hyposegmented – can appear as a single round nucleus (unilobed, homozygous Pelger-Huet Anomaly) or dumbbell shaped (bilobed, heterozygous Pelger-Huet Anomaly). Anomaly is differentiated from a left shift by displaying mature chromatin pattern, abundant cytoplasm (low nuclear:cytoplasmic ratio), mature granulation, and an absence of toxic changes.
Congenital Pelger-Huet: granulocytes show normal granulation, 50-90% of neutrophils are affected.
Pseudo Pelger-Huet: seen in leukocyte malignancies and Myelodysplastic Syndrome, hypogranulation and other Dy’s plastic features may be present, 10-30% of neutrophils are affected.
Clinical Significance and Cause:1,3,5
Pelger-Huet Anomaly is benign and cell function is normal. Psuedo Pelger-Huet may indicate leukocyte malignancies and myelodysplasia.
Congenital: Lamin β-receptor gene mutation.
Acquired (Pseudo-Pelger-Huet): Hematologic malignancies such as myelodysplastic syndrome (MDS), acute myeloid leukemia (AML), myeloproliferative neoplasms (MPNs). Pseudo-Pelger-Huet may also be seen during infections, and drug interactions.
Inheritance Pattern:1-3,5
Autosomal dominant
CBC:2
Congenital Pelger-Huet: Cytopenias often absent
Pseudo-Pelger-Huet: Cytopenias often present
References:
1. Turgeon ML. Nonmalignant Disorders of Granulocytes and monocytes. In: Clinical hematology: theory and procedures. 4th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 1999. p. 206-16.
2. Cunningham JM, Patnaik MM, Hammerschmidt DE, Vercellotti GM. Historical perspective and clinical implications of the Pelger-Huet cell. Am J Hematol [Internet]. 2009 Oct 20 [cited 2018 Jul 10];84(2):116–9. Available from: https://doi.org/10.1002/ajh.21320
3. Manonneaux S. Nonmalignant leukocyte disorders. In: Rodak’s hematology clinical applications and principles. 5th ed. St. Louis, Missouri: Saunders; 2015. p. 475-97.
4. Harmening DM, Marty J, Strauss RG. Cell biology, disorders of neutrophils, infectious mononucleosis, and reactive lymphocytosis. In: Clinical hematology and fundamentals of hemostasis. 5th ed. Philadelphia: F.A. Davis Company; 2009. p. 305-30.
5. Landis-Piwowar K. Nonmalignant disorders of leukocytes: granulocytes and monocytes. In: Clinical laboratory hematology. 3rd ed. New Jersey: Pearson; 2015. p. 388-407.