May-Hegglin anomaly

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anomaly

 [ah-nom´ah-le]
marked deviation from normal. adj., adj anom´alous.
Axenfeld's anomaly a developmental anomaly characterized by a circular opacity of the posterior peripheral cornea, and caused by an irregularly thickened, axially displaced Schwalbe's ring.
congenital anomaly (developmental anomaly) absence, deformity, or excess of body parts as the result of faulty development of the embryo.
Ebstein's anomaly see ebstein's anomaly.
May-Hegglin anomaly a rare dominantly inherited disorder of blood cell morphology, characterized by RNA-containing cytoplasmic inclusions (similar to Döhle bodies) in granulocytes, by large, poorly granulated platelets, and by thrombocytopenia.

Hegg·lin a·nom·a·ly

(heg'lin),
a disorder in which neutrophils and eosinophils contain basophilic structures known as Döhle or Amato bodies and in which there is faulty maturation of platelets, with thrombocytopenia; autosomal dominant inheritance.
Synonym(s): May-Hegglin anomaly

May-Hegglin anomaly

a rare autosomal-dominant inherited blood cell disorder characterized by thrombocytopenia and granulocytes with blue-colored ribonucleic acid containing cytopathic inclusions similar to Döhle's bodies.
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May-Hegglin anomaly

May-Hegglin anomaly

A rare autosomal dominant condition (OMIM:155100) characterised by the triad of thrombocytopenia, giant platelets and Döhle-body-like inclusions in WBC cytoplasm.

Clinical findings
Recurrent epistaxis, gingival bleeding, easy bruising, menorrhagia, excess bleeding post-surgery.
 
Management
Most patients are asymptomatic; corticosteroids, splenectomy are ineffective; platelet transfusions may be needed.

May-Hegglin anomaly

Hematology A rare AD condition characterized by the triad of thrombocytopenia, giant platelets, and Döhle-body-like inclusions in WBC cytoplasm Clinical Recurrent epistaxis, gingival bleeding, easy bruising, menorrhagia, excess bleeding postsurgery Management Most Pts are asymptomatic; corticosteroids, splenectomy are useless; platelet transfusions may be needed. See Hereditary thrombocytopenia.

Hegglin,

Robert M.P., Swiss internist, 1907-1970.
Hegglin anomaly - a disorder in which neutrophils and eosinophils contain Döhle bodies and in which there is faulty maturation of platelets, with thrombocytopenia. Synonym(s): May-Hegglin anomaly
Hegglin syndrome - an energy-dynamic cardiac insufficiency during diabetic coma and other metabolic disorders. Synonym(s): May-Hegglin syndrome
May-Hegglin anomaly - Synonym(s): Hegglin anomaly
May-Hegglin syndrome - Synonym(s): Hegglin syndrome

May,

Richard, German physician, 1863-1936.
May-Hegglin anomaly - Synonym(s): Hegglin anomaly
May-Hegglin syndrome - Synonym(s): Hegglin syndrome
References in periodicals archive ?
Mutations of MYH9, encoding for non-muscle myosin heavy chain A, in May Hegglin anomaly.