A rare familial thrombocytopenia: May-Hegglin anomaly report of two cases and review of the literatureHüseyin Gülen1, Ayşe Erbay2, Elif Kazancı2, Canan Vergin21Deartment Of Pediatric Hematology, Celal Bayar University, School Of Medicine, Manisa, Turkey
2Dr. Behcet Uz Children's Hospital, Hematology-oncology, İzmir, Turkey
May-Hegglin anomaly is a hereditary thrombocytopenia associated with giant platelets and large basophilic, cytoplasmic inclusion bodies (resembling Döhle bodies) in the granulocytes. Patients may experience easy bruising, recurrent epistaxis, gingival bleeding, menorrhagia and sometimes excessive bleeding associated with surgical procedures. Failure to appropriately diagnose May-Hegglin anomaly could result in inappropriate treatment. In states of chronic thrombocytopenia associated with large platelets, including chronic idiopathic thrombocytopenic purpura, May-Hegglin anomaly should be considered in the differential diagnosis. In this case report, we present a five-year-old girl previously followed as idiopathic thrombocytopenic purpura without bleeding symptoms and a 14-year-old boy who were diagnosed with May-Hegglin anomaly. Keywords: Thrombocytopenia, May-Hegglin
Nadir bir trombositopeni nedeni: May-Hegglin anomalili iki olgu sunusu ve literatür özetiHüseyin Gülen1, Ayşe Erbay2, Elif Kazancı2, Canan Vergin21Department Of Pediatric Hematology, Celal Bayar University, School Of Medicine, Manisa
2Dr. Behcet Uz Children's Hospital, Hematology-oncology, İzmir
May-Hegglin anomalisi dev trombositler ve granülositik hücrelerde Döhle cisimciklerine benzer büyük bazofilik inklüzyon cisimciklerinin varlığıyla karakterize nadir bir kalıtsal trombsitopenidir. Kolay morarma, tekrarlayan burun kanamaları, dişeti kanamaları, menoraji ve bazen özellikle cerrahi girişimler sırasında aşırı kanama gibi belirtiler verebilir. Doğru tanı konulamaması uygunsuz tedavilerin kulanılmasına yol açabilir. Dev trombositler ve kronik trombositopeni birlikteliğinde kronik idiopatik trombositopenik purpura yanısıra May-Hegglin anomalisi de ayırıcı tanıda düşünülmelidir. Bu olgu sunusunda; önceden kanama semptomları olmayan ancak idiopatik trombositopenik purpura tanısı konmuş 5 yaşında kız ve 14 yaşında erkek hasta sunulmaktadır. Anahtar Kelimeler: Trombositopeni, May-Hegglin
Hüseyin Gülen, Ayşe Erbay, Elif Kazancı, Canan Vergin. A rare familial thrombocytopenia: May-Hegglin anomaly report of two cases and review of the literature. Turk J Hematol. 2006; 23(2): 111-114
Corresponding Author: Hüseyin Gülen, Türkiye |
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