Mesut GARİPARDİÇ, Sultan KABA, Nihat DEMİR, Erdal PEKER, Oğuz TUNCER

Glanzmann Thrombasthenia Mimicking Early Neonatal Sepsis

Glanzmann trombastenisi pıhtı oluşumunun ilk basamağındaki anormalikten kaynaklanan otozomal resesif geçişli kalıtsal bir hastalıktır. Ekimotik mukokütenöz leziyonlarla ve kana diateziyle ilişkili olgularda, eğer trombosit sayısı normal, kanama zamanı uzun ise Glanzamann trombastenisi ayırıcı tanıda düşünülmelidir. Burada ikiz eşi olarak doğan, diğer ikizi intauterin exitus olan ve yaygın intravasküler koagulopati ve sepsis tablosuyla gelen ardından GT tanısı konan bir olgu sunuldu.

Erken Neonatal Sepsisi Andıran bir Glanzman Trombasteni Olgusu

Glanzmann thrombasthenia is an autosomal and recessively inherited disorder resulting from abnormality in the first step of thrombosis. In cases associated with hemorrhagic diathesis and ecchymotic mucocutaneous lesions, Glanzmann thrombasthenia should be taken into account in differential diagnosis if thrombocyte count is normal and bleeding duration is too long. In this study, we present the case of a pair of twins in which one of the siblings was lost in the uterus while the other had sepsis along with common intravascular coagulopathy and was eventually diagnosed with Glanzmann thrombasthenia.

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