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DOI: 10.1055/s-0038-1644876
HEREDITARY PLATELET FUNCTION DISORDERS IN JAPAN
Publication History
Publication Date:
23 August 2018 (online)
Nationwide surveys of hereditary platelet function disorders in Japan were carried out in 1976, 1981, and 1986. Information on 271 cases received in the 1986 survey was analyzed with that for 103 other cases reported in earlier surveys but not in 1986, making a total of 374 cases. The mortality rate was 6.8% of 162 cases in 1976, 6.6% of 213 cases in 1981, and 5.9% of 374 cases in 1986. Bleeding symptoms appeared at age 12 years in 56.8% of patients and the most common were epistaxis and purpura. Of the 295 cases 49.5% were isolated cases, 20.3% had siblings with confirmed bleeding tendencies, and 30.2% had other kin with bleeding tendencies, suggestings autosomal tnansmission. Consanguineous marriage was reported by 11.9% of patients.
Of the 374 cases in 1986, 59.4% were thrombasthenia (TA), 11.5% Bernard-Soulier symdrome (BSS), 22.5% release abnormalities (PRA), 1.3% other, and 5.3% unclassified. Of the 84 cases of PRA, 60 were storage pool deficiency, 18 release mechanism abnormalities, and 6 undecided between the two types. The resultsof laboratory tests were as follows .