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The rare dense bodies were much bigger than normally observed; their density was diminished and was localized in a peripheral ring. Other platelet constituents were found to be normal. Platelet peroxidase activity was normal in the canaliculi of the dense tubular system; catalase\u2010positive granules were also present. Serotonin uptake by the patient's platelets was much decreased and reserpine, a potent inhibitor of serotonin accumulation by normal human platelets, did not further decrease this incorporation. The uptake of free <jats:sup>14<\/jats:sup>C\u2010arachidonic acid by the platelets was greatly diminished, as was its thrombin\u2010induced liberation from phosphatidylcholine and phosphatidyl inositol. Moreover, platelet phospholipase A<jats:sub>1<\/jats:sub> activity was much reduced and phospholipase A<jats:sub>2<\/jats:sub> activity was undetectable.<\/jats:p>","DOI":"10.1002\/ajh.2830040410","type":"journal-article","created":{"date-parts":[[2007,2,21]],"date-time":"2007-02-21T05:19:22Z","timestamp":1172035162000},"page":"387-399","source":"Crossref","is-referenced-by-count":32,"title":["Studies on a new variant of the Hermansky\u2010Pudlak syndrome: Qualitative, ultrastructural, and functional abnormalities of the platelet\u2010dense bodies associated with a phospholipase a defect"],"prefix":"10.1002","volume":"4","author":[{"given":"Francine","family":"Rendu","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jeanine","family":"Breton\u2010Gorius","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Germain","family":"Trugnan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hugo","family":"Castro\u2010Malaspina","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jean\u2010Marie","family":"Andrieu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gilbert","family":"Bereziat","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marilyne","family":"Lebret","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jacques P.","family":"Caen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2006,7,11]]},"reference":[{"key":"e_1_2_1_2_2","first-page":"163","article-title":"Albinism associated with hemorrhagic diathesis and unusual pigmented reticular cells in the bone marrow: Report of two cases with histochemical studies","volume":"4","author":"Hermansky F","year":"1959","journal-title":"Blood"},{"key":"e_1_2_1_3_2","first-page":"413","article-title":"Thrombopathies h\u00e9r\u00e9ditaires par trouble de lib\u00e9ration","volume":"16","author":"Hermansky F","year":"1976","journal-title":"Nouv Rev Fr H\u00e9mat"},{"key":"e_1_2_1_4_2","first-page":"319","article-title":"Studies of platelets in a variant of the Hermansky\u2010Pudlak syndrome","volume":"63","author":"White JG","year":"1971","journal-title":"Am J Pathol"},{"key":"e_1_2_1_5_2","first-page":"590","article-title":"Ultrastructural features of abnormal blood platelets. 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