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Acute myeloid leukaemia (AML) with t(6;11)(q27;q23), resulting in a chimeric MLL/AF6 gene, is rare (≤0.6% of adult cases),1 ,2 and is associated with poor prognosis.2 ,3 The majority of these cases are acute myelomonocytic leukaemia, or acute monoblastic/monocytic leukaemia,1 and they have increased risk for hyperleukocytosis requiring leukapheresis.4 The RAS association domain (RA1) at the N-terminus of AF6 is sufficient for MLL-AF6 to mediate leukemogenesis.5 Although an activated KRAS mutation accelerates MLL/AF4-driven leukemogenesis in transgenic mice,6 whether it will do the same with MLL/AF6 is unknown. Here, we report a rare and aggressive case of acute monocytic leukaemia with both MLL/AF6 and KRAS G12D.
A middle-aged man in his 40s had sore throat, chills, night sweats and swelling in the neck for four days prior to presentation to a local emergency centre. Concurrently, non-pruritic purple skin lesions appeared on his body. A complete blood count showed a white blood cell (WBC) count of 3 25 000/mm3, a platelet count of 60 000/mm3, and 92% monocytes in the blood smear. He was given 6 g of hydroxyurea orally and transferred to a tertiary cancer centre for emergent treatment of hyperleukocytosis. His past medical history, social history and family history were non-contributory.
Upon arrival at the cancer centre, he became dyspneic. His vital signs were: blood pressure 107/53 mm Hg; heart rate …
Contributors All authors participated in the evaluation and management of this case, acquisition of data or analysis and interpretation of data, drafting and revising the manuscript. All authors approved the version of the manuscript being submitted.
Competing interests None.
Provenance and peer review Not commissioned; externally peer reviewed.