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Review Article
Hematology
Genetic classification and confirmation of inherited platelet disorders: current status in Korea
Ye Jee Shim
Clin Exp Pediatr. 2020;63(3):79-87.   Published online February 6, 2020
Inherited platelet disorders (IPDs), which manifest as primary hemostasis defects, often underlie abnormal bleeding and a family history of thrombocytopenia, bone marrow failure, hematologic malignancies, undefined mucocutaneous bleeding disorder, or congenital bony defects. Wide heterogeneity in IPD types with regard to the presence or absence of thrombocytopenia, platelet dysfunction, bone marrow failure, and dysmegakaryopoiesis is observed in patients. The individual...
Case Report
A Case of Type Ⅰ Glanzmann's Thrombasthenia Diagnosed by Flow Cytometry
Mun Su Lee, Choong Ho Shin, Kyu Young Kim, Dong Woo Son, Hwa Ryung Chung, Do Hyun Kim
Clin Exp Pediatr. 1999;42(1):133-137.   Published online January 15, 1999
Glanzmann's thrombasthenia is a rare autosomal recessive hemorrhagic disorder of platelet function with missing or abnormal platelet plasma membrane glycoprotein Ⅱb-Ⅲa, which functions as a receptor for fibrinogen. We have experienced a case of thrombasthenia in a 6-year-old female whose chief complaints were easy bruising, frequent epistaxis, arthralgia and swelling of the right ankle joint. Bleeding time was prolonged in...
Original Article
Analysis of Platelet Membrane Glycoprotein Ⅱb-Ⅲa complex in Whole Blood of Glanzmann's Thrombasthenia by Flow Cytometry
Byoung Geun Lee, Man Choon Kang, Jong Man Park, Pyung Han Hwang, Jung Soo Kim
Clin Exp Pediatr. 1994;37(11):1540-1547.   Published online November 15, 1994
Glanzmann's thrompasthenia is a rare autosomal recessive hemorrhagic disorder characterized by prolonged bleeding time, and deficient or absent clot retraction in the presence of normal platelet count. The major underlying abnormality in this disease is grossly defective first-phase aggregation of platelet, which are unresponsive to ADP or other platelet agonists such as epinephrine, collagen, thrombin in any concentration. This disability...
A case of thrombasthenia.
Yong Bae Suh, Eun Kyoung Sohn, Yong Mook Choi, Chang Il Ahn
Clin Exp Pediatr. 1991;34(2):287-291.   Published online February 28, 1991
Glanzmann’s thrombasthenia is a congenital disorder of platelet function with missing or abnormal platelet plasma membrane glycoprotein Ilb/IIIa which function as receptors for fibrinogen and other high-molecular-weight adhesive proteins which are important for platelet aggregation. We have experienced a case of thrombasthenia in a 12 month old male whose chief complaints were easy bruising and frequent epistaxis. The beeding time was prolonged in the...
Two cases of Glanzmann's thrombasthenia in brother.
Won Mo Yang, Hyung Kook Ham, Mee Kyung Namgoong, Baek Keun Lim, Hong Sup Yoon, Kab Joon Yoon
Clin Exp Pediatr. 1989;32(11):1574-1580.   Published online November 30, 1989
Glanzmann’s thrombasthenia is rare congenital bleeding disorder of autosomal recessive trait. We experienced two cases of Glanzmann’s thrombasthenia in brother. The mother and elder brother had no history of bleeding tendency. But 10 year-old and 7 year-old younger brothers had frequent epistaxis, gum bleeding and easy bruisability. On platelet aggregation test, mother and elder brother appeared normal responses, but the younger brothers appeared no responses...
Case Report
Three Cases of Glanzmann's Thrombasthenia.
Sung In Baek, Myung Chul Hyun, Haeng Mi Kim, Kun Soo Lee, Doo Hong Ahn, Hyo Jin Chun, Jay Sik Kim
Clin Exp Pediatr. 1985;28(11):1153-1157.   Published online November 30, 1985
We experienced 3 cases of Glanzmann' s thrombasthenia of 2 year-old boy, 4 year-old boy, and 5 year-old girlt who represented with frequent epistaxis and petechia. The later two of them are brother and sister.Laboratory findings were characterized by normal platelet counts, markedly diminished clot retraction, prolonged bleeding time, positive Rumpel-Leede test, abnormal platelet adhesion to glass, and complete failure...