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Related Experiment Videos

Gray platelet syndrome in the elderly.

A Berrebi1, A Klepfish, D Varon

  • 1Hematology Unit, Kaplan Hospital, Rehovot, Israel.

American Journal of Hematology
|August 1, 1988
PubMed
Summary

Gray platelet syndrome, a rare alpha-granule deficiency, can present in adults. This case demonstrates a 68-year-old male with prolonged survival despite this rare bleeding disorder.

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Area of Science:

  • Hematology
  • Genetics
  • Pathology

Background:

  • Gray platelet syndrome is an extremely rare inherited bleeding disorder.
  • It is characterized by a deficiency of platelet alpha-granules, affecting platelet function and leading to hemorrhagic diathesis.

Observation:

  • A 68-year-old male presented with a 18-year history of thrombocytopenia and mild splenomegaly.
  • Peripheral blood smear revealed agranular gray platelets, and bone biopsy showed mild reticular fibrosis.
  • Electron microscopy confirmed a near-complete absence of platelet alpha-granules, with absent thrombospondin and fibronectin.

Findings:

  • The patient exhibited hemorrhagic diathesis, including ecchymoses and post-operative bleeding requiring platelet transfusions.
  • Follow-up over 4 years showed stable parameters with no evidence of myeloproliferative or dysmyelopoietic disorders.

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  • The findings suggest a diagnosis of constitutional primary alpha-granule deficiency or gray platelet syndrome.

    • Implications:

    • This case highlights that gray platelet syndrome, typically described in very young patients, can have a long and relatively uneventful survival in adults.
    • It underscores the importance of considering rare genetic platelet disorders in patients with unexplained bleeding tendencies.
    • Further research into the long-term management and genetic basis of gray platelet syndrome is warranted.