Briefly, the important clinical findings were as follows: A 10 year old white girl, acutely ill, febrile and showing disseminated skin purpura. There was no visceromegaly. The disease had apparently started 3 weeks before, with epistaxis and other purpuric manifestations. Laboratory findings showed severe anemia and thrombocytopenia with a leukocyte count of 3500 per cu. mm. The differential co...

Sai-Juan Chen, * Arthur Zelent, * Jian-Hua Tong, * Huai-Qin Yu, * Zhen-Yi Wang, * Josette Derre,1 Roland Berger,' Samuel Waxman,11 and Zhu Chen* *Shanghai Institute ofHematology, Rui-Jin Hospital, Shanghai Second Medical University, and Shanghai Central Railway Hospital, Shanghai 200025, China; *Leukaemia Research Fund Center at the Institute ofCancer Research, Chester Beatty Laboratories, Fulh...

1. Zelent A, Guidez F, Melnick A, Waxman S, Licht JD. Translocations of the RARα gene in acute promyelocytic leukemia. Oncogene 2001;20:7186-203. 2. Catalano A, Dawson MA, Somana K, Opat S, Schwarer A, Campbell LJ, et al. The PRKAR1A gene is fused to RARA in a new variant acute promyelocytic leukemia. Blood 2007;110:4073-6. 3. Kwok C, Zeisig BB, Dong S, So CW. Forced homooligomerization of RARα...

Tightly regulated expression of the transcription factor PU.1 is crucial for normal hematopoiesis. PU.1 knockdown mice develop acute myeloid leukemia (AML), and PU.1 mutations have been observed in some populations of patients with AML. Here we found that conditional expression of promyelocytic leukemia-retinoic acid receptor alpha (PML-RARA), the protein encoded by the t(15;17) translocation f...

BACKGROUND The secondary genetic changes other than the promyelocytic leukemia-retinoic acid receptor (PML-RARA) fusion gene may contribute to the acute promyelocytic leukemogenesis. Chromosomal alterations and mutation of FLT3 (FMS-like tyrosine kinase 3) tyrosine kinase receptor are the frequent genetic alterations in acute myeloid leukemia. However, the prognostic significance of FLT3 mutati...

Since the first description of acute promyelocytic leukemia (APL) in 1957 as the most malignant form of acute leukemia, several developments have paved the way to make this disease the most curable leukemia in adults and change the paradigm of cancer treatment. Therapy of APL was pioneered by Bernard et al in 1973 demonstrating a striking sensitivity to daunorubicin, probably related to signifi...