May-Hegglin anomaly

Alternative eponyms

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A cytoplasmic leukocyte anomaly characterized by the presence Döhle or Amato bodies (2-5 µ) in neutrophils and eosinophils, thrombocytopenia, and giant blood platelets.

Description

A cytoplasmic leukocyte anomaly characterized by the presence Döhle or Amato bodies (2-5 µ) in neutrophils and eosinophils, thrombocytopenia, and giant blood platelets. Usually the condition is asymtomatic, but about one-fourth have a hemorrhagic tendency which is related to thrombocytopenia and abnormal platelet function. Döhle bodies are usually found in the neutrophils, but they may also occur in eosinophils, basophils, monocytes, and lymphocytes. A rare disorder with familial characteristics, inherited as an autosomal dominant trait.

May first described the white cell changes in 1909 and Hegglin recognised the associated platelet anomaly in 1945.

Hegglin’s patients included a father and two sons, aged 24 and 14 years. Döhle bodies were present in myelocytes from sternal marrow. Despite persistent thrombocytopenia, increased capillary fragility, and delayed clot retraction, these patients continued to remain in good health. A study of 31 aunts, uncles, and cousins revealed no similar hematologic abnormalities.

Bibliography

  • R. May:
    Leukocyteneinschlüsse. Kasuistische Mitteilung.
    Deutsches Archiv für klinische Medizin, Leipzig, 1909, 96: 1-6.
  • R. Hegglin:
    Über eine neue Form einer konstitutionellen Leukozytenanomalie, kombiniert mit Throbopathie.
    Schweizerische medizinische Wochenschrift, Basel, 1945, 75: 91-92. Gleichzeitige konstitutionelle Veränderungen an Neutrophilen und Thrombozyten.
    Helvetica medica acta, Basel, 1945, 12: 439-440.

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