Disease Information for May-Hegglin anomaly

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Clinical Manifestations
Disease Progression
Course/Chronic only
Demographics & Risk Factors
Population Group
Child
Population/Pediatrics population
Sex & Age Groups
Population/Child
Population/Child-Infant Only
Population/Children/all
Laboratory Tests
Abnormal Lab Findings (Non Measured)
Bleeding time prolonged/platelet count normal (Lab)
Giant abnormal platelets/smear (Lab)
Granulocyte Inclusions
Platelets large on smear (Lab)
Platelets, atypical/on smear (Lab)
Abnormal Lab Findings - Decreased
Platelet count (Lab)
Abnormal Lab Findings - Increased
Bleeding time, Duke (Lab)
Associated Diseases & Rule outs
Associated Disease & Complications
May-Hegglin Anomaly
Thrombocytopenia
Thrombocytopenia in Children
Disease Mechanism & Classification
Class
CLASS/Hematologic (category)
CLASS/Leukocyte involvement/disorder (ex)
CLASS/Marrow involvement/disorder (ex)
Process
PROCESS/Congenital/developmental (category)
PROCESS/Hereditary platelet disorders (ex)
Definition

May Hegglin Anomaly; Dohle Leukocyte Inclusions with Giant Platelets; Dohle"s Bodies-Myelopathy; Hegglin"s Disease; Leukocytic Inclusions with Platelet Abnormality; MHA; Macrothrombocytopenia with Leukocyte Inclusions; May-Hegglin Anomaly is a rare, inherited, blood platelet disorder characterized by abnormally large and misshapen platelets (giant platelets) and defects of the white blood cells known as leukocytes; The defect of the white blood cells consists of the presence of very small (2-5 micrometers) rods, known as Dohle bodies, in the fluid portion of the cell (cytoplasm); Some people with this disorder may have no symptoms while others may have various bleeding abnormalities; In mild cases, treatment for May-Hegglin Anomaly is not usually necessary; In more severe cases, transfusions of blood platelets may be necessary; In the past couple of years, it has become clear to physicians studying this disorder that May-Hegglin Anomaly is one of a family of five autosomal dominant, giant platelet disorders, each of which involves slight variants (alleles) of the same gene in the same location; The other giant platelet disorders related to May-Hegglin Anomaly are Sebastian Syndrome, Fechtner Syndrome, Epstein Syndrome, and the Alport-like Syndrome with macrothrombocytopenia; [NORD 2005 ]-------------------------------Hegglin"s anomaly; A disorder in which neutrophils and eosinophils contain basophilic structures known as Dohle or Amato bodies and in which there is faulty maturation of platelets, with thrombocytopenia; autosomal dominant inheritance; Synonym: May-Hegglin anomaly; [Dorland]--------------------------------------------May-Hegglin anomaly

Hegglin’s anomaly;Hegglin’s syndrome;May-Hegglin syndrome;s:

Döhle body-panmyelopathic syndrome; Döhle bodies-myelopathy syndrome;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; first cases: Despite persistent thrombocytopenia, increased capillary fragility, and delayed clot retraction, these patients continued to remain in good health-------------[whonamedit_com 2005]--------

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NEJM (The New England Journal of Medicine)
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