TY - JOUR
T1 - Spontaneous regression of aleukemic leukemia cutis harboring a NPM/RARA fusion gene in an infant with cutaneous mastosytosis
AU - Kanegane, Hirokazu
AU - Nomura, Keiko
AU - Abe, Akihiro
AU - Makino, Teruhiko
AU - Ishizawa, Shin
AU - Shimizu, Tadamichi
AU - Naoe, Tomoki
AU - Miyawaki, Toshio
PY - 2009/1
Y1 - 2009/1
N2 - Aleukemic leukemia cutis has been rarely reported in infant leukemia. This report describes a 6-month-old boy with aleukemic leukemia cutis, which regressed without any treatments within 6 months. Interestingly, a cytogenetic analysis disclosed a leukemia clone with the karyotype of 46, XY, t(5;17)(q35;q12), which generated nucleophosmin (NPM)-retinoic acid receptor α fusion (RARA) fusion transcripts. The patient simultaneously had cutaneous mastocytosis, which also disappeared with the leukemia cutis. He shows no physical or laboratory abnormalities without any treatments after 12 months, although the NPM/RARA transcripts remain faintly in the bone marrow. The present case is partially compatible with systemic mastocytosis with an associated clonal hematologic non-mast cell lineage disorder, proposed by the WHO classification, and it is also suggestive of the initiation or early stage of acute promyelocytic leukemia.
AB - Aleukemic leukemia cutis has been rarely reported in infant leukemia. This report describes a 6-month-old boy with aleukemic leukemia cutis, which regressed without any treatments within 6 months. Interestingly, a cytogenetic analysis disclosed a leukemia clone with the karyotype of 46, XY, t(5;17)(q35;q12), which generated nucleophosmin (NPM)-retinoic acid receptor α fusion (RARA) fusion transcripts. The patient simultaneously had cutaneous mastocytosis, which also disappeared with the leukemia cutis. He shows no physical or laboratory abnormalities without any treatments after 12 months, although the NPM/RARA transcripts remain faintly in the bone marrow. The present case is partially compatible with systemic mastocytosis with an associated clonal hematologic non-mast cell lineage disorder, proposed by the WHO classification, and it is also suggestive of the initiation or early stage of acute promyelocytic leukemia.
KW - Aleukemic leukemia cutis
KW - Mastocytosis
KW - Nucleophosmin
KW - Retinoic acid receptor α
UR - http://www.scopus.com/inward/record.url?scp=67349203316&partnerID=8YFLogxK
U2 - 10.1007/s12185-008-0216-y
DO - 10.1007/s12185-008-0216-y
M3 - 学術論文
C2 - 19052694
AN - SCOPUS:67349203316
SN - 0925-5710
VL - 89
SP - 86
EP - 90
JO - International Journal of Hematology
JF - International Journal of Hematology
IS - 1
ER -