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European Journal of Pediatrics

, Volume 151, Issue 9, pp 668–671 | Cite as

May-Hegglin anomaly: A rare cause of thrombocytopenia

  • A. Greinacher
  • J. Bux
  • V. Kiefel
  • J. G. White
  • C. Mueller-Eckhardt
Hematology/Oncology

Abstract

A family with four and an unrelated family with three individuals affected by the May-Hegglin anomaly are described. Platelet counts were markedly reduced and were correctly determined only in the counting chamber. Bleeding time and platelet aggregation were always normal, but platelet nucleotide concentrations (ATP and ADP) were elevated. The platelet glyco-protein complexes Ib/IX, IIb/IIIa and Ia/IIa were quantitatively normal. Platelet-associated IgG was slightly elevated, although thrombocytopenia was presumably not caused by an immunological mechanism. Morphological investigations showed giant platelets and spindle-shaped inclusion bodies in the granulocytes, while their function (phagocytic capacity, radical production) was normal. To exclude hereditary types of thrombocytopenia, morphological and family investigations are required to avoid misdiagnosis with far-reaching diagnostic and therapeutic consequences.

Key words

Thrombocytopenia Giant platelets Leucocytes May-Hegglin anomaly Hereditary thrombocytopenia 

Abbreviation

PRP

platelet-rich plasma

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Copyright information

© Springer-Verlag 1992

Authors and Affiliations

  • A. Greinacher
    • 1
  • J. Bux
    • 1
  • V. Kiefel
    • 1
  • J. G. White
    • 2
  • C. Mueller-Eckhardt
    • 1
  1. 1.Institute for Clinical Immunology and Transfusion MedicineJustus Liebig UniversityGiessenGermany
  2. 2.Departments of Pathology, Laboratory Medicine and PaediatricsUniversity of MinnesotaMinneapolisUSA

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