Medich giant platelet disorder: a unique alpha granule deficiency I. Structural abnormalities

Platelets
James G White

Abstract

Human platelet granule deficiency disorders include the gray platelet syndrome (GPS), alpha delta storage pool deficiency, the Hermansky-Pudlak syndrome and the White platelet syndrome. The present study describes a patient with a lifelong history of easy bleeding, thrombocytopenia and giant platelets. Her cells were found to have normal numbers of dense bodies, but a markedly decreased number of alpha granules. Many platelets had no alpha granules and resembled the gray platelets of patients with GPS. However, the empty vacuoles without granule contents that fill the cytoplasm of GPS platelets were not present in significant numbers in her platelets. In addition to the decrease in alpha granules the patients platelets contained membranous inclusions resembling cigars or scrolls. Usually, only one scroll open at each end was present, but many platelets contained two and some as many as five. Freeze-fracture revealed an absence of intramembranous particles in many layers of the scrolls. They occur in no other human platelet disorder, but are common in platelets from the Wistar-Furth rat. Thus, the patient is a unique variant of human platelet granule deficiency disorders unlike any described previously.

References

Nov 1, 1978·Molecular and Cellular Biochemistry·J G White, J M Gerrard
Jan 1, 1990·Advances in Human Genetics·J G White
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Mar 19, 2002·Platelets·James G WhiteAndreas Greinacher

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Citations

Jul 19, 2012·The Anatomical Record : Advances in Integrative Anatomy and Evolutionary Biology·Olivia J ScullyGeorge W Yip
Aug 4, 2011·Journal of Thrombosis and Haemostasis : JTH·A Nurden, P Nurden
Aug 31, 2012·Platelets·William GunningJames G White
Jan 31, 2006·Blood Reviews·Alan T Nurden, Paquita Nurden
May 20, 2009·Blood Reviews·Price Blair, Robert Flaumenhaft
Mar 31, 2021·British Journal of Haematology·Janine CollinsDragana Vuckovic

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