Spontaneous improvement of hematologic abnormalities in patients having juvenile myelomonocytic leukemia with specific RAS mutations

被引：121

作者：

Matsuda, Kazuyuki ^{[1
]}

Shimada, Akira ^{[2
]}

Yoshida, Nao ^{[3
]}

Ogawa, Atsushi ^{[4
]}

Watanabe, Akihiro ^{[4
]}

Yajima, Shuhei ^{[5
]}

Iizuka, Susumu ^{[6
]}

Koike, Kazutoshi ^{[7
]}

Yanai, Fumio ^{[8
]}

Kawasaki, Keiichiro ^{[9
]}

Yanagimachi, Masakatsu ^{[10
]}

Kikuchi, Akira ^{[14
]}

Ohtsuka, Yoshitoshi ^{[11
]}

Hidaka, Eiko ^{[1
]}

Yamauchi, Kazuyoshi ^{[1
]}

Tanaka, Miyuki ^{[12
]}

Yanagisawa, Ryu ^{[12
]}

Nakazawa, Yozo ^{[12
]}

Shiohara, Masaaki ^{[12
]}

Manabe, Atsushi ^{[13
]}

Kojima, Seiji ^{[3
]}

Koike, Kenichi ^{[12
]}

机构：

[1] Shinshu Univ Hosp, Dept Lab Med, Matsumoto, Nagano, Japan

[2] Gunma Childrens Med Ctr, Dept Hematol Oncol, Gunma, Japan

[3] Nagoya Univ, Grad Sch Med, Dept Pediat, Nagoya, Aichi, Japan

[4] Niigata Canc Ctr Hosp, Dept Pediat, Niigata, Japan

[5] Hamamatsu Med Ctr, Hamamatsu, Shizuoka, Japan

[6] Hokkaido Canc Ctr, Dept Pediat, Sapporo, Hokkaido, Japan

[7] Ibaraki Childrens Hosp, Dept Pediat, Mito, Ibaraki, Japan

[8] Fukuoka Univ, Sch Med, Dept Pediat, Fukuoka 81401, Japan

[9] Hyogo Prefectural Kobe Childrens Hosp, Dept Hematol & Oncol, Kobe, Hyogo, Japan

[10] Yokohama City Univ, Sch Med, Dept Pediat, Yokohama, Kanagawa 232, Japan

[11] Hyogo Coll Med, Dept Pediat, Nishinomiya, Hyogo, Japan

[12] Shinshu Univ, Sch Med, Dept Pediat, 3-1-1 Asahi, Matsumoto, Nagano 3908621, Japan

[13] St Lukes Int Hosp, Dept Pediat, Tokyo, Japan

[14] Saitama Childrens Med Ctr, Div Hematol Oncol, Saitama, Japan

来源：

BLOOD | 2007年 / 109卷 / 12期

关键词：

D O I：

10.1182/blood-2006-09-046649

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Of 11 children with juvenile myelomonocytic leukemia (JMML) carrying RAS mutations (8 with NRAS mutations, 3 with KRAS2 mutations), 5 had a profound elevation in either or both the white blood cells and spleen size at diagnosis. Three patients had no or modest hepatosplenomegaly and mild leukocytosis at presentation but subsequently showed a marked increase in spleen size with or without hematologic exacerbation, for which nonintensive chemotherapy was initiated. The other three patients with NRAS or KRAS2 glycine to serine substitution received no chemotherapy, but hematologic improvement has been observed during a 2- to 4-year follow up. In the third group, all hematopoletic cell lineages analyzed had the RAS mutations at the time of hematologic improvement, whereas DNA obtained from the nails had the wild type. Additionally, numbers of circulating granulocyte-macrophage progenitors were significantly reduced during the clinical course. Thus, some patients with JMMIL with specific RAS mutations may have spontaneously improving disease.

引用

页码：5477 / 5480

页数：4

共 21 条

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