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Natural cytotoxicity impairment in familial haemophagocytic lymphohistiocytosis.

作者信息

Aricò M, Nespoli L, Maccario R, Montagna D, Bonetti F, Caselli D, Burgio G R

机构信息

Department of Paediatrics, University of Pavia, Italy.

出版信息

Arch Dis Child. 1988 Mar;63(3):292-6. doi: 10.1136/adc.63.3.292.

Abstract

Ten children with the characteristic clinical and haematological features of haemophagocytic lymphohistiocytosis are reported. Four patients treated with a combination of drugs comprising etoposide, methotrexate, and steroids were in complete remission after 10 to 30 months. Natural cytotoxic mechanisms including natural killer cell activity, antibody dependent cell mediated cytotoxicity, lymphokine activated killer cell activity, and natural killer cell like activity were persistently absent or severely impaired in these four patients despite their clinical remission. Their parents and one healthy sibling also had impaired natural cytotoxic mechanisms. Constitutional impairment of natural cytotoxic mechanisms could be important in the pathogenesis of haemophagocytic lymphohistiocytosis.

摘要

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本文引用的文献

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Familial haemophagocytic reticulosis.家族性噬血细胞性网状细胞增生症
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2
Successful treatment of lymphohistiocytic reticulosis with phagocytosis with epipodophyllotoxin VP 16-213.
Cancer. 1980 May 15;45(10):2516-20. doi: 10.1002/1097-0142(19800515)45:10<2516::aid-cncr2820451008>3.0.co;2-v.
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Genetic analysis of familial erythrophagocytic lymphohistiocytosis.
Eur J Pediatr. 1984 Sep;142(4):248-52. doi: 10.1007/BF00540245.
7
Heterogeneity of natural killer cells.自然杀伤细胞的异质性。
Annu Rev Immunol. 1984;2:359-94. doi: 10.1146/annurev.iy.02.040184.002043.
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Impaired natural killer activity in lymphohistiocytosis syndrome.
J Pediatr. 1984 Apr;104(4):569-73. doi: 10.1016/s0022-3476(84)80549-1.
9
Familial hemophagocytic lymphohistiocytosis.家族性噬血细胞性淋巴组织细胞增生症
Eur J Pediatr. 1983 Jun-Jul;140(3):221-30. doi: 10.1007/BF00443367.

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