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1 & 1/2 -year-old girl is sent to a children's hospital for evaluation following a nosebleed which was so severe as to require nasal packing and transfusion of platelet concentrates. When a blood sample had been drawn in the emergency room for serum chemistry studies, the local hospital laboratory had noted that the clot that formed was unusual in that it failed to retract. Peripheral blood smear obtained by finger puncture showed an appropriate number of normal-sized platelets, all of which were individual, without clumping. At the children's hospital, it was noted that the child's parents were cousins. Special platelet studies showed that the child's platelet's failed to aggregate with any physiologic aggregating agent, including a high concentration of exogenous ADP. Which of the following is the most likely diagnosis?

A. Bernard-Soulier syndrome
B. Chediak-Higashi syndrome
C. May-Hegglin anomaly
D. Thrombasthenia
E. Von Willebrand disease




D ??????




Glanzmann thrombasthenia defect in platelet membrane glycoprotein IIb-IIIa complex..
Hence the answer is D. Thrombasthenia

Differentiate it from
A. Bernard-Soulier syndrome
Giant platelets and thrombocytopenia abnormality in Gp Ib




grin Thanks ARJ..

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