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Golgi complexes in hypogranular platelet syndromes.

James G White1

  • 1Department of Laboratory Medicine and Pathology, University of Minnesota, Minneapolis, MN 55455, USA. white003@umn.edu

Platelets
|March 15, 2005
PubMed
Summary

White platelet syndrome (WPS) is unique in its high number of Golgi complexes in circulating platelets. Other inherited platelet disorders, like gray platelet syndrome (GPS), do not share this specific characteristic.

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Area of Science:

  • Hematology
  • Cell Biology
  • Genetics

Background:

  • White platelet syndrome (WPS) is an inherited disorder causing hypogranular platelets.
  • WPS platelets contain more than 13% of fully developed Golgi complexes from megakaryocytes.

Purpose of the Study:

  • To investigate if Golgi complex perpetuation in circulating platelets is common in other hypogranular platelet disorders.
  • To determine if WPS's characteristic Golgi complex presence is unique.

Main Methods:

  • Comparative analysis of Golgi complex frequency in platelets from patients with various inherited hypogranular platelet disorders.
  • Microscopic examination of circulating platelets to quantify Golgi complexes.

Main Results:

  • Platelets from gray platelet syndrome (GPS) and one alpha delta platelet storage pool deficiency (alpha delta SPD) patient showed increased Golgi complexes.
  • Hermansky-Pudlak syndrome, other alpha delta SPD cases, and Medich giant platelet disorder platelets had normal Golgi complex frequencies.

Conclusions:

  • The high number of circulating Golgi complexes observed in white platelet syndrome (WPS) appears to be a unique characteristic.
  • Perpetuation of Golgi complexes is not a common link across all evaluated hypogranular platelet disorders.

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