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Atypical mast cells (CD2+, CD25+, CD117+) with toluidine blue metachromasia were found in the peripheral blood and on bone marrow aspiration smears. Chemotherapy with cytosine arabinoside and idarubicin was ineffective and the patient died of multi\u2010organ failure with rapidly progressing hepatosplenomegaly and large\u2010volume ascites 3\u2003months after admission. At autopsy the bone marrow, spleen, liver, and lymph nodes were extensively infiltrated by atypical tumor cells with occasional bi\u2010 or multi\u2010lobated nuclei. They were positive for mast cell tryptase and possessed an activating mutation of the <jats:italic>c\u2010kit<\/jats:italic>gene (D816V). Ascites (2200\u2003mL) and non\u2010ruptured esophageal varices with submucosal hemorrhage indicated the presence of severe portal hypertension. Although there was no evidence of liver cirrhosis, the hepatic sinusoids were clogged with tumor cells, with a tendency to be more severe in the perivenular areas, and the lumens of central veins were obliterated by tumor cell infiltration. The present case demonstrates that non\u2010cirrhotic portal hypertension due to blocking of sinusoidal and venous flow could be a serious complication in mast cell leukemia.<\/jats:p>","DOI":"10.1111\/j.1440-1827.2009.02451.x","type":"journal-article","created":{"date-parts":[[2009,10,27]],"date-time":"2009-10-27T04:31:07Z","timestamp":1256617867000},"page":"817-822","source":"Crossref","is-referenced-by-count":7,"title":["Mast cell leukemia with rapidly progressing portal hypertension"],"prefix":"10.1111","volume":"59","author":[{"given":"Masayuki","family":"Yoshida","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yuji","family":"Nishikawa","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yohei","family":"Yamamoto","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yuko","family":"Doi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takuo","family":"Tokairin","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Toshiaki","family":"Yoshioka","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yasufumi","family":"Omori","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Atsushi","family":"Watanabe","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Naoto","family":"Takahashi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tomoko","family":"Yoshioka","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ikuo","family":"Miura","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ken\u2010ichi","family":"Sawada","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Katsuhiko","family":"Enomoto","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2009,10,27]]},"reference":[{"key":"e_1_2_5_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0145-2126(01)00038-8"},{"key":"e_1_2_5_3_2","first-page":"54","volume-title":"World Health Organization Classification of Tumours of Hematopoietic and Lymphoid Tissues","author":"Horny HP","year":"2008"},{"key":"e_1_2_5_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2004.02.045"},{"key":"e_1_2_5_5_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2009-02-205237"},{"key":"e_1_2_5_6_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0025-6196(12)62636-6"},{"key":"e_1_2_5_7_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830340409"},{"key":"e_1_2_5_8_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00277-007-0430-3"},{"key":"e_1_2_5_9_2","doi-asserted-by":"publisher","DOI":"10.1080\/10428190412331272695"},{"key":"e_1_2_5_10_2","doi-asserted-by":"publisher","DOI":"10.1002\/hep.1840220423"},{"key":"e_1_2_5_11_2","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(19890201)63:3<532::AID-CNCR2820630322>3.0.CO;2-F"},{"key":"e_1_2_5_12_2","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(86)90622-4"},{"key":"e_1_2_5_13_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.92.23.10560"},{"key":"e_1_2_5_14_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0145-2126(01)00041-8"},{"key":"e_1_2_5_15_2","doi-asserted-by":"publisher","DOI":"10.1080\/10428190400010775"},{"key":"e_1_2_5_16_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1980.tb03805.x"},{"key":"e_1_2_5_17_2","doi-asserted-by":"publisher","DOI":"10.1097\/00000478-200307000-00019"},{"key":"e_1_2_5_18_2","doi-asserted-by":"publisher","DOI":"10.1016\/0016-5085(78)90303-7"},{"key":"e_1_2_5_19_2","first-page":"76","article-title":"Systemic mastocytosis with portal hypertension. 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