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
Main Authors 奥田, 敏徳, 岩崎, 沙理, 土居, 忠, 蟹沢, 祐司, 石津, 明洋, 田中, 信悟, 黄, 勇澤, 太田, 英敏, 藤見, 章仁, 佐藤, 康裕
Format Journal Article
LanguageJapanese
Published 一般社団法人 日本内科学会 2009
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Online AccessGet full text
ISSN0021-5384
1883-2083
DOI10.2169/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.
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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Snippet 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...
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SubjectTerms chronic eosinophilic leukemia
FIP1L1/PDGFRα
imatinib
spontaneous pneumothorax
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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