The Pelger-Huët anomaly. A clinical and ultrastructural study of a family from northeastern Mexico

N. Sotelo-Cruz, A. González-Olivas, G. Covarrubias-Espinoza, J. L. Lira Garcés, P. Valencia-Mayoral

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Abstract

The Pelger-Huet anomaly is a benign disorder which affects the morphology of the granulocytes and is namely inherited as an autosomic dominant trait. The frequency of this anomaly varies from country to country and in México this constitutes the sixth family reported. This is a case of a 21-month-old boy whose anomaly was detected during the study of his ferropenic anemia with which he arrived at the hospital. During the study of his nine family members, the anomaly was found to have also been present in his father. An electron microscopy study of the subject, his father's and a normal control's neutrophils was conducted. The patient's and his father's leucocyte granules were found to be normal but decreased in number. This work includes some discussion on inherited traits, their presentation, morphology, differential diagnosis and emphasizes the importance in recognizing this anomaly and the promotion of genetic counselling.
Original languageAmerican English
Pages (from-to)261-266
Number of pages6
JournalBoletin Medico del Hospital Infantil de Mexico
StatePublished - 1 Apr 1991
Externally publishedYes

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