SEVERE JUVENILE VAGINAL BLEEDING DUE TO GLANZMANNS-THROMBASTHENIA - CASE-REPORT AND REVIEW OF THE LITERATURE

Glanzmann's thrombasthenia is a rare inherited hematological disorder defined by deficiency or abnormality of the glycoprotein (GP) IIb-IIIa complex, Presenting symptoms are hemorrhagic events, mainly epistaxis , purpura, or menorrhagia, We describe the clinical course and managem ent of a 14-year-old girl with Glanzmann's thrombasthenia and severe m enorrhagia, Following treatment with 20 U of packed red blood cells, 3 7 U of platelets, 7 U of fresh frozen plasma, cryoprecipitate, intrave nous estrogens, and methylergotrine maleate with no improvement, the u terine cavity was packed for 48 hr. This unusual procedure halted the bleeding and avoided the necessity for a hysterectomy, When treating a cute menorrhagia in patients with Glanzmann's thrombasthenia, the phys ician should be familiar with the characteristics and all treatment mo dalities for this disorder. (C) 1998 Wiley-Liss, Inc.