Abstract
FISH-negative, cytogenetically cryptic acute promyelocytic leukemia.
Highlights
600–800 new cases of acute promyelocytic leukemia (APL) occur annually in the United States.1 The advent of all-trans retinoic acid (ATRA) has converted this subtype of acute leukemia to a readily curable one with excellent long-term outcomes
Immediate initiation of ATRA upon morphological and clinical suspicion of APL is recommended to prevent the typical and often devastating bleeding diathesis associated with APL, definitive diagnosis relies on the demonstration of PML-RARA translocation
The diagnosis in such cases can be established by reverse transcriptase PCR (RT-PCR), the clinical challenge in fluorescence in situ hybridization (FISH)-negative, cytogenetically cryptic APL is in making a decision on continuation of ATRA before the diagnosis is definitively established
Summary
Blood Cancer Journal (2015) 5, e320; doi:10.1038/bcj.2015.47; published online 19 June 2015. Death (ED; within 30 days of hospitalization) occurred in 3 (18%) of the 17 patients for whom outcome data were available One of these patients received ATRA-based induction and the other two died before treatment initiation. Failure to make the correct diagnosis can have a catastrophic outcome This is important given our observation that 85% of patients had laboratory and/or clinical evidence of DIC at presentation. Recognition of suggestive morphology and typical clinical and laboratory findings in FISH-negative, cytogenetically cryptic cases and immediate initiation of ATRA followed by its continuation until RT-PCR results are available are key to successful treatment. ATRA/ATO underutilization (10% of patients received chemotherapy alone) and demonstrate the need for improvement in clinical and morphological diagnosis of APL
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