Glanzmann Thrombasthenia Mimicking Early Neonatal Sepsis

Öz

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.

Key Word: Glanzmann; Sepsis; Hemorrhagic Diathesis.

Anahtar Kelimeler

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NihatDemir1, Sultan Kaba2, Mesut Garipardiç2, Erdal Peker1, Oğuz Tuncer1

Öz

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

Anahtar Kelimeler

• Glanzman, Sepsis, Kanama Diyatezi

Kaynakça

1. Glanzmann E. Hereditaire hemorrhagische thrombasthenie. Ein Beitrag zur pathologie der blutplattchen j Kinderkranken 1918;88:113.
2. Neunert CE, Journeycake JM: Congenital platelet disorders. Hematol Oncol Clin North Am 2007; 21:663-84.
3. Israels SJ, Kahr WH, Blanchette VS, Luban NL, Rivard GE, Rand ML: Platelet disorders in children: A diagnostic approach. Pediatr Blood Cancer 2011; 56:975-83.
4. Sebastiano C, Bromberg M, Breen K, Hurford MT. Glanzmann's thrombasthenia: report of a case and review of the literature. Int J Clin Exp Pathol 2010;25;443-7.
5. Kutlubay B, Özdemir GN, Tüysüz G, Apak H, Celkan T. Glanzmann thrombasthenia: Cerrahpaşa Medical Faculty experience. Türk Ped Arfl 2012;47:104-6.
6. Zenciroglu A, Bas AY, Demirel N, Yarali N. Glanzmann thrombasthenia in a neonate. Indian Pediatr 2007;44:40-2.
7. Fışgın T, Koca D. Kalıtsal Trombosit Hastalıkları. Türk Çocuk Hematoloji Dergisi 2011;5:1-10.
8. Karl WM. Intrauterine death in a twin: implications for the survivor. In: Ward RH, Whittle M, editors. Multiple pregnancy. London: RCOG Pres 1995;218-30.

9. Blickstein I, Perlman S. Single fetal death in twin gestations. J Perinat Med 2013;41:65-9.