TY - JOUR
T1 - FLT3-internal tandem duplication in a pediatric patient with t(8;21) acute myeloid leukemia
AU - Kawamura, Machiko
AU - Kaku, Hidefumi
AU - Ito, Tateki
AU - Funata, Nobuaki
AU - Taki, Tomohiko
AU - Shimada, Akira
AU - Hayashi, Yasuhide
N1 - Funding Information:
We would like to thank all members of the Hematology Division in the Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital for joining in this patient's care, and also Janet E. Lewis (University of Wisconsin-Madison) for helping with the preparation of the manuscript. This study was supported by the Clinical Research Foundation of Tokyo Metropolitan Hospital and the Clinical Cancer Research Foundation of Sumitomo Trust Bank-charitable trust team .
PY - 2010/12
Y1 - 2010/12
N2 - Patients diagnosed with t(8;21)-acute myeloid leukemia (AML) are currently considered to have good prognoses, but about half of these patients relapse. FLT3-internal tandem duplication (ITD) is generally thought to be strongly associated with poor prognosis in AML, but is rarely reported in patients with t(8;21)-AML. Expression of the neural cell-adhesion molecule (CD56) is also associated with a significantly shorter complete remission duration and survival in patients with t(8;21)-AML. Patients with t(8;21)-AML expressing CD56 have been reported to exhibit a higher incidence of granulocytic sarcoma (GS), and t(8;21)-AML with GS results in a less favorable prognosis than AML with this translocation alone. Here, we report on a 15-year-old girl with t(8;21)-AML having both CD56 expression and FLT3-ITD. This patient underwent unrelated donor bone marrow transplantation and achieved complete remission, but thereafter presented with obstructive jaundice caused by GS compression of the common bile duct without bone marrow invasion at relapse. Autopsy revealed multiple nodules of the stomach membrane and invasion into the head of the pancreas. For earlier detection of relapse, we suggest that it would be useful to examine existence of GS in CD56-positive t(8;21)-AML patients at diagnosis and hematologic remission. Even though t(8;21)-AML is less likely to co-occur with FLT3-ITD in pediatric patients, this report suggests that prognostic factors, including FLT3 and KIT genes and the surface marker CD56, should be analyzed in these patients.
AB - Patients diagnosed with t(8;21)-acute myeloid leukemia (AML) are currently considered to have good prognoses, but about half of these patients relapse. FLT3-internal tandem duplication (ITD) is generally thought to be strongly associated with poor prognosis in AML, but is rarely reported in patients with t(8;21)-AML. Expression of the neural cell-adhesion molecule (CD56) is also associated with a significantly shorter complete remission duration and survival in patients with t(8;21)-AML. Patients with t(8;21)-AML expressing CD56 have been reported to exhibit a higher incidence of granulocytic sarcoma (GS), and t(8;21)-AML with GS results in a less favorable prognosis than AML with this translocation alone. Here, we report on a 15-year-old girl with t(8;21)-AML having both CD56 expression and FLT3-ITD. This patient underwent unrelated donor bone marrow transplantation and achieved complete remission, but thereafter presented with obstructive jaundice caused by GS compression of the common bile duct without bone marrow invasion at relapse. Autopsy revealed multiple nodules of the stomach membrane and invasion into the head of the pancreas. For earlier detection of relapse, we suggest that it would be useful to examine existence of GS in CD56-positive t(8;21)-AML patients at diagnosis and hematologic remission. Even though t(8;21)-AML is less likely to co-occur with FLT3-ITD in pediatric patients, this report suggests that prognostic factors, including FLT3 and KIT genes and the surface marker CD56, should be analyzed in these patients.
UR - http://www.scopus.com/inward/record.url?scp=78649981249&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=78649981249&partnerID=8YFLogxK
U2 - 10.1016/j.cancergencyto.2010.07.130
DO - 10.1016/j.cancergencyto.2010.07.130
M3 - Article
C2 - 21156247
AN - SCOPUS:78649981249
VL - 203
SP - 292
EP - 296
JO - Cancer Genetics and Cytogenetics
JF - Cancer Genetics and Cytogenetics
SN - 0165-4608
IS - 2
ER -