@article{oai:u-ryukyu.repo.nii.ac.jp:02015960,
 author = {野崎, 宏幸 and Nozaki, Hiroyuki},
 issue = {3},
 journal = {琉球医学会誌 = Ryukyu Medical Journal},
 month = {},
 note = {Hemostatic disorders are classified as abnormalities of platelets, abnormalities of coagulation factors, and abnormalities of blood vessels. Platelets are derived from megakaryocytes in bone marrow. Adhesion, aggregation and release-reactions are major functions of platelets. Patients with platelet disorders have symptoms such as bruising or mucosal bleeding. Idiopathic thrombocytopenic purpura (ITP) is an autoimmune disorder characterized by platelet destruction due to an antiplatelet autoantibody. Corticosteroids are recommended as initial treatment in ITP patients. However, persistent response rates are not so high. Persistent complete and partial response rates were 14% and 34%, respectively in our study. Splenectomy results in a much higher cure rate than any medical regimen. In our study, splenectomy induced a stable complete response in 56% of ITP patients having no permanent unmaintained response to steroids. Immunosuppression, intravenous $ \gamma $-globulin, danazol, ascorbic acid, and intravenous methylprednisolone have been used in ITP patients who fail steroids and splenectomy. In our study, any ITP patients did not achieve normal platelet levels when treated with low dose of danazol or ascorbic acid, though long term ascorbic acid therapy appeared to be slightly effective in aged ITP patients. Thrombotic thrombocytopenic purpura (TTP) is a heterogenous clinical syndrome characterized by the pentad of thrombocytopenic purpura, hemolytic anemia, neurologic abnormalities, fever and renal dysfunction. The etiology of TTP is not fully clarified. The survival rate has increased to about 80% with the use of plasma exchange. In our experience, a TTP patient entered persistent remission by the treatment of splenectomy after plasma exchange. The patients with thrombocytopenia due to underproduction require platelet transfusions to control bleeding. In our previous study, 45% of patients with acute leukemia and 89% of patients with aplastic anemia developed alloimmunization due to leukocyte contamination in transfusion products. Therefore, reducing the level of leukocyte contamination is necessary. Thrombasthenia is now defined as a hemorrhagic diathesis associated with a prolonged bleeding time, a normal platelet count, and absent macroscopic platelet aggregation in response to ADP or other platelet agonists. The biochemical lesion responsible for the disorder is a deficiency of the platelet membrane glycoprotein IIb/IIIa complex. Bernard-Soulier syndrome and storage pool disease also belong to congenital qualitative platelet disorders. Many drugs affect platelet functions. Especially, aspirin inhibits platelets responses irreversibly by inactivation of the enzyme cyclooxygenase. Some patients with chronic myeloproliferative disorders have acquired qualitative platelet abnormalities., 論文},
 pages = {107--111},
 title = {[総説］血小板異常を伴う血液疾患},
 volume = {15},
 year = {1995}
}