Alpha-Storage Pool Deficiency of Platelet in a Patient with Valvular Heart Disease / 대한혈액학회지
Korean Journal of Hematology
;
: 421-426, 1998.
Article
in Korean
| WPRIM
| ID: wpr-720412
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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Aortic Valve
/
Autoimmune Diseases
/
Thrombocytopenia
/
Biopsy
/
Bleeding Time
/
Blood Platelets
/
Bone Marrow
/
Platelet Storage Pool Deficiency
/
Megakaryocytes
/
Microscopy, Electron
Limits:
Adult
/
Humans
/
Male
Country/Region as subject:
Asia
Language:
Korean
Journal:
Korean Journal of Hematology
Year:
1998
Type:
Article
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