The platelet count threshold for World Health Organization classification system-compliant diagnosis of essential thrombocythemia (ET) is ?450 × 109/L.1 Extreme thrombocytosis (ExT), on the other hand, arbitrarily defined by ?1000 109/L and occurs in approximately 22% patients with ET, at time diagnosis.2 Note, ExT most frequent context young ET incidence rates 44%/29%/29% aged ?40, 41–60, > 60...

概要 二 次的栓球 増多を きたす 明 らか な原因疾患が な く,著 明 な持続的栓球増 多 と血栓 お よび出血 傾 向を示す疾患 は,1920年Diguglielmoがpiastrinemiaと して報 告 したのを は じめ として,い ろいろな 名称 を冠 した本症 の報告 が散見 され る.著 者 らは,64才 の男子 で,本 症 と診断 され る1例 を 経験 し た.本 症例 は,両 側足趾 に次 々 と疼痛 ・壊死が出現 し,時 に,鼻 出血 ・歯 肉出血 をみ た.末 梢血 中の 栓球数は ほ とん ど常 に100万/mm3を 越 え,最 高238万/mm3に 達 し,軽 度 の貧血 と白血球増 多を伴つ た.骨 髄所見 では,骨 髄 巨核球が237/mm3と 増加 し,ま た,栓 球生成能 の亢進が示唆 された.し か し,赤 ・白血球系細胞 には異常 をみ な...

Polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF) are categorized as myeloproliferative neoplasms (MPNs). All are characterized by the autonomous growth of one or more lineages of hematopoietic cells, splenomegaly, constitutional symptoms such as fatigue, night sweating, itching, and weight loss, and is frequently complicated by thrombosis and hemorrhage. J...

Diagnosis of paraneoplastic skin syndromes associating neoplastic processes is assumed as the crucial aspect of dermatological practice. Knowledge of clinical findings of dermatoses suggesting coincidence of malignant proliferative processes facilitates diagnostic and therapeutic procedures. We would like to present a case of Sweet's syndrome, qualified for comparative paraneoplastic skin syndr...

Essential thrombocythemia (ET) is an indolent myeloproliferative neoplasm that may be complicated by vascular events, including both thrombosis and bleeding. This disorder may also transform into more aggressive myeloid neoplasms, in particular into myelofibrosis. The identification of somatic mutations of JAK2, CALR, or MPL, found in about 90% of patients, has considerably improved the diagnos...