好酸球の広範な肺浸潤により気胸を呈したimatinib耐性化慢性好酸球性白血病の剖検例
近年,FIP1L1/PDGFRα融合遺伝子(FP遺伝子)陽性の慢性好酸球性白血病(chronic eosinophilic leukemia;CEL)に対するimatinib(IM)の有効性が示され,治療成績は著しく改善しているが長期予後には不明な点も多い.今回,我々はIMに耐性化し終末期に自然気胸を併発したCEL症例を経験した.剖検で好酸球の肺胞への高度の浸潤およびその近傍の線維化,気腫化が確認され,CELの進行が肺実質の破壊をきたし気胸の原因となったと推定された....
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Published in | 日本内科学会雑誌 Vol. 98; no. 4; pp. 862 - 865 |
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Main Authors | , , , , , , , , , |
Format | Journal Article |
Language | Japanese |
Published |
一般社団法人 日本内科学会
2009
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Subjects | |
Online Access | Get full text |
ISSN | 0021-5384 1883-2083 |
DOI | 10.2169/naika.98.862 |
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Abstract | 近年,FIP1L1/PDGFRα融合遺伝子(FP遺伝子)陽性の慢性好酸球性白血病(chronic eosinophilic leukemia;CEL)に対するimatinib(IM)の有効性が示され,治療成績は著しく改善しているが長期予後には不明な点も多い.今回,我々はIMに耐性化し終末期に自然気胸を併発したCEL症例を経験した.剖検で好酸球の肺胞への高度の浸潤およびその近傍の線維化,気腫化が確認され,CELの進行が肺実質の破壊をきたし気胸の原因となったと推定された. |
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AbstractList | 近年,FIP1L1/PDGFRα融合遺伝子(FP遺伝子)陽性の慢性好酸球性白血病(chronic eosinophilic leukemia;CEL)に対するimatinib(IM)の有効性が示され,治療成績は著しく改善しているが長期予後には不明な点も多い.今回,我々はIMに耐性化し終末期に自然気胸を併発したCEL症例を経験した.剖検で好酸球の肺胞への高度の浸潤およびその近傍の線維化,気腫化が確認され,CELの進行が肺実質の破壊をきたし気胸の原因となったと推定された. |
Author | 土居, 忠 田中, 信悟 藤見, 章仁 佐藤, 康裕 太田, 英敏 岩崎, 沙理 石津, 明洋 蟹沢, 祐司 奥田, 敏徳 黄, 勇澤 |
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References | 1) 藤見章仁, 他: FIP1L1-PDGFRα融合遺伝子とTCR再構成がみられImatinibにより寛解に至ったLymphomatoid Papulosisを伴う好酸球増多症候群の1例. 内科学会雑誌 96: 2794-2797, 2007. 5) Yellin A, et al: Pneumothorax associated with lymphoma. Am Rev Respir Dis 134: 590-592, 1986. 8) Bobnoff N, et al: Myeloid blast crisis evolving during imatinib treatment of an FIP1L1-PDGFR alpha-positive chronic myeloproliferative disease with prominent eosinophilia. Leukemia 19: 286-289, 2005. 7) Cools J, et al: A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 gene as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome. N Engl J Med 348: 1201-1214, 2003. 9) Ohnishi H, et al: Chronic eosinophilic leukemia with FIP1L1-PDGFRA fusion and T674I mutation that evolved from Langerhans cell histiocytosis with eosinophilia after chemotherapy. Br J Haematol 134: 544-549, 2006. 3) Steinhauslin CA, et al: Spontaneous pneumothorax. A complication of lung cancer? Chest 88: 709-713, 1985. 4) O'Connor BM, et al: Spontaneous pneumothorax in small cell lung cancer. Chest 102: 628-629, 1992. 6) Geltner D, et al: Acute lymphocytic leukemia. Its occurrence with hypereosinophilic syndrome and bilateral spontataneous pneumothorax. Arch Intern Med 138: 292-293, 1978. 10) Hingorani SR, et al: Targeting oncogene dependence and resistance. Cancer Cell 3: 414-417, 2003. 2) Dines DE, et al: Malignant pulmonary neoplasms predisposing to spontaneous pneumothorax. Mayo Clin Proc 48: 541-544, 1973. |
References_xml | – reference: 9) Ohnishi H, et al: Chronic eosinophilic leukemia with FIP1L1-PDGFRA fusion and T674I mutation that evolved from Langerhans cell histiocytosis with eosinophilia after chemotherapy. Br J Haematol 134: 544-549, 2006. – reference: 6) Geltner D, et al: Acute lymphocytic leukemia. Its occurrence with hypereosinophilic syndrome and bilateral spontataneous pneumothorax. Arch Intern Med 138: 292-293, 1978. – reference: 2) Dines DE, et al: Malignant pulmonary neoplasms predisposing to spontaneous pneumothorax. Mayo Clin Proc 48: 541-544, 1973. – reference: 3) Steinhauslin CA, et al: Spontaneous pneumothorax. A complication of lung cancer? Chest 88: 709-713, 1985. – reference: 1) 藤見章仁, 他: FIP1L1-PDGFRα融合遺伝子とTCR再構成がみられImatinibにより寛解に至ったLymphomatoid Papulosisを伴う好酸球増多症候群の1例. 内科学会雑誌 96: 2794-2797, 2007. – reference: 4) O'Connor BM, et al: Spontaneous pneumothorax in small cell lung cancer. Chest 102: 628-629, 1992. – reference: 5) Yellin A, et al: Pneumothorax associated with lymphoma. Am Rev Respir Dis 134: 590-592, 1986. – reference: 10) Hingorani SR, et al: Targeting oncogene dependence and resistance. Cancer Cell 3: 414-417, 2003. – reference: 8) Bobnoff N, et al: Myeloid blast crisis evolving during imatinib treatment of an FIP1L1-PDGFR alpha-positive chronic myeloproliferative disease with prominent eosinophilia. Leukemia 19: 286-289, 2005. – reference: 7) Cools J, et al: A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 gene as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome. N Engl J Med 348: 1201-1214, 2003. |
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Title | 好酸球の広範な肺浸潤により気胸を呈したimatinib耐性化慢性好酸球性白血病の剖検例 |
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