Alpha-Storage Pool Deficiency of Platelet in a Patient with Valvular Heart Disease.
- Author:
Byung Hak JUNG
1
;
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
;
Hyun Sook CHI
Author Information
1. Department of Medicine, Asan Medical Center, College of Medicine, University of Ulsan, Seoul, Korea.
- Publication Type:Case Report
- Keywords:
Platelet;
Storage pool deficiency;
Valvular heart disease
- MeSH:
Adult;
Aortic Valve;
Autoimmune Diseases;
Biopsy;
Bleeding Time;
Blood Platelets*;
Bone Marrow;
Cardiopulmonary Bypass;
Cell Membrane;
Collagen;
Disseminated Intravascular Coagulation;
Dyspnea;
Epinephrine;
Heart Valve Diseases*;
Hemorrhage;
Humans;
Hyperplasia;
Male;
Megakaryocytes;
Microscopy, Electron;
Mitral Valve;
Mitral Valve Insufficiency;
Myeloproliferative Disorders;
Platelet Aggregation;
Platelet Storage Pool Deficiency;
Platelet Transfusion;
Republic of Korea;
Thrombocytopenia
- From:Korean Journal of Hematology
1998;33(3):421-426
- CountryRepublic of Korea
- Language:Korean
-
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.
