Korean J Hematol 1998; 33(3):
Published online September 30, 1998
© The Korean Society of Hematology
심판막 질환에 병발한 혈소판의 알파-저장푸울 결핍증 1례
정병학, 서철원, 김상희, 김태원, 윤환중, 장대영, 최종수, 이제환, 김성배, 김상위, 이규형, 이정신, 김우건, 박찬정, 지현숙
울산대학교 의과대학 서울중앙병원 내과학교실,
울산대학교 의과대학 서울중앙병원 임상병리학교실
Alpha-Storage Pool Deficiency of Platelet in a Patient with Valvular Heart Disease
Alpha-storage pool deficiency of platelet is a very rare disorder associated with a variety of conditions, including autoimmune disease, disseminated intravascular
coagulation, myeloproliferative disorders, and cardiopulmonary bypass. This bleeding disorder is characterized by a moderate thrombocytopenia and a selective abnormality in platelet alpha-granules.
We experienced the first case of alpha-storage pool deficiency of platelet in a 28-year-old male with severe valvular heart disease in Republic of Korea. Two years
ago, mitral valve replacement was performed in other university hospital. Since a few months ago, dyspnea was developed and progressively exacerbated. Transesophageal
echocardiogram showed severe mitral valve regurgitation and aortic valve regurgitation. He had moderate thrombocytopenia. Bleeding time was prolonged. Peripheral blood smear showed hypogranular platelets with indistinct cell membrane. In bone marrow biopsy, there showed evidence of mild hyperplasia of megakaryocytes. Platelet aggregation test revealed abnormal response to collagen and epinephrine. Electron microscopy of periphral blood showed the vacuolization of alpha-granules of platelets. Following platelet transfusion, double valve replacement could be performed successfully. Six months later, platelet morphology was moderately normalized. But bleeding time and platelet aggregation test were still abnormal.
Keywords Platelet; Storage pool deficiency; Valvular heart disease;
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Korean J Hematol 1998; 33(3): 421-426
Published online September 30, 1998
Copyright © The Korean Society of Hematology.
심판막 질환에 병발한 혈소판의 알파-저장푸울 결핍증 1례
정병학, 서철원, 김상희, 김태원, 윤환중, 장대영, 최종수, 이제환, 김성배, 김상위, 이규형, 이정신, 김우건, 박찬정, 지현숙
울산대학교 의과대학 서울중앙병원 내과학교실,
울산대학교 의과대학 서울중앙병원 임상병리학교실
Alpha-Storage Pool Deficiency of Platelet in a Patient with Valvular Heart Disease
Byung Hak Jung, Cheolwon Suh, Sang Hee Kim, Tae Won Kim, Whan Jung Yoon, Dae Young Zang, Jong Soo Choi, Je Hwan Lee, Sung Bae Kim, Kyoo Hyung Lee, Jung Shin Lee, Woo Kun Kim, Chan Jeoung Park, Kyun Sook Chi
Department of Medicine, Asan Medical Center, Clinical Pathology, College of Medicine, University of Ulsan, Seoul, Korea
Abstract
Alpha-storage pool deficiency of platelet is a very rare disorder associated with a variety of conditions, including autoimmune disease, disseminated intravascular
coagulation, myeloproliferative disorders, and cardiopulmonary bypass. This bleeding disorder is characterized by a moderate thrombocytopenia and a selective abnormality in platelet alpha-granules.
We experienced the first case of alpha-storage pool deficiency of platelet in a 28-year-old male with severe valvular heart disease in Republic of Korea. Two years
ago, mitral valve replacement was performed in other university hospital. Since a few months ago, dyspnea was developed and progressively exacerbated. Transesophageal
echocardiogram showed severe mitral valve regurgitation and aortic valve regurgitation. He had moderate thrombocytopenia. Bleeding time was prolonged. Peripheral blood smear showed hypogranular platelets with indistinct cell membrane. In bone marrow biopsy, there showed evidence of mild hyperplasia of megakaryocytes. Platelet aggregation test revealed abnormal response to collagen and epinephrine. Electron microscopy of periphral blood showed the vacuolization of alpha-granules of platelets. Following platelet transfusion, double valve replacement could be performed successfully. Six months later, platelet morphology was moderately normalized. But bleeding time and platelet aggregation test were still abnormal.
Keywords: Platelet, Storage pool deficiency, Valvular heart disease,
