Alder-Reilly Anomaly in the Cerebrospinal Fluid of a Child With Hurler Syndrome

Alder-Reilly Anomaly in the Cerebrospinal Fluid of a Child With Hurler Syndrome Hurler syndrome is an autosomal recessive mucopolysaccharidosis characterized by intralysosomal accumulation of glycosaminoglycan fragments, with cellular accumulation of distended lysosomes resulting in interference with normal cell function. One of the peripheral blood features of mucopolysaccharidoses is the presence of numerous, dark lilac granules within lymphocytes, monocytes, and neutrophils, also known at Alder-Reilly anomaly. Here we describe intracytoplasmic granules with haloes in mononuclear cells present in the cerebrospinal fluid of a 2-year-old boy with the diagnosis of Hurler syndrome, undergoing pretransplant evaluation for an unrelated donor cord blood stem cell transplant. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Journal of Pediatric Hematology / Oncology Wolters Kluwer Health

Alder-Reilly Anomaly in the Cerebrospinal Fluid of a Child With Hurler Syndrome

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Publisher
Wolters Kluwer
Copyright
Copyright © 2017 Wolters Kluwer Health, Inc. All rights reserved.
ISSN
1077-4114
eISSN
1536-3678
D.O.I.
10.1097/MPH.0000000000001041
Publisher site
See Article on Publisher Site

Abstract

Hurler syndrome is an autosomal recessive mucopolysaccharidosis characterized by intralysosomal accumulation of glycosaminoglycan fragments, with cellular accumulation of distended lysosomes resulting in interference with normal cell function. One of the peripheral blood features of mucopolysaccharidoses is the presence of numerous, dark lilac granules within lymphocytes, monocytes, and neutrophils, also known at Alder-Reilly anomaly. Here we describe intracytoplasmic granules with haloes in mononuclear cells present in the cerebrospinal fluid of a 2-year-old boy with the diagnosis of Hurler syndrome, undergoing pretransplant evaluation for an unrelated donor cord blood stem cell transplant.

Journal

Journal of Pediatric Hematology / OncologyWolters Kluwer Health

Published: Jan 1, 2018

References

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