9897/3 Acute myloid leukemia, 11q23 abnormalities


Synonyms

Acute myloid leukemia, MLL
Acute myeloid leukemia with t(9;11)(p22;q23); MLLT3-MLL

Definitions

Tumours of haematopoietic and lymphoid tissues
ICD-O-3 topography code: C42 and C77
ICD10: C92.0
           

/span>cute myeloid leukaemia with t(9;11) (p22;q23); MLLT3-MLL is usually associated with monocytic features.

> n adult and paediatric acute leukaemia, with over 50 translocation partner genes now characterized 1
Click to access Pubmed
Meyer C, Schneider B, Jakob S, Strehl S, Attarbaschi A, Schnittger S, Schoch C, Jansen MW, van Dongen JJ, den Boer ML, Pieters R, Ennas MG, Angelucci E, Koehl U, Greil J, Griesinger F, Zur Stadt U, Eckert C, Szczepa?ski T, Niggli FK, Schäfer BW, Kempski H, Brady HJ, Zuna J, Trka J, Nigro LL, Biondi A, Delabesse E, Macintyre E, Stanulla M, Schrappe M, Haas OA, Burmeister T, Dingermann T, Klingebiel T, Marschalek R (2006)
The MLL recombinome of acute leukemias.
Leukemia 20: 777-84



2
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Shih LY, Liang DC, Fu JF, Wu JH, Wang PN, Lin TL, Dunn P, Kuo MC, Tang TC, Lin TH, Lai CL (2006)
Characterization of fusion partner genes in 114 patients with de novo acute myeloid leukemia and MLL rearrangement.
Leukemia 20: 218-23



. Translocations involving MLLT2 (AF4), resulting predominantly in acute lymphoblastic leukaemia (ALL), and MLLT3 (AF9), resulting predominantly in AML, are the most common. Other MLL translocations that commonly result in AML include the MLLT1 (ENL), MLLT10 (AF10), MLLT4 (AF6) and ELL as partner genes. Other than the MLL-ELL fusion ?resulting from the t(11;19)(q23;p13.1), which is most often associated with only AML, these fusions occur predominantly in AML, but may be seen in ALL as well. Up to one third of MLL translocations in AML are not detectable on conventional karyotype analysis, and FISH or other ?molecular studies may be necessary to identify these variant translocations 3
Click to access Pubmed
Shih LY, Liang DC, Fu JF, Wu JH, Wang PN, Lin TL, Dunn P, Kuo MC, Tang TC, Lin TH, Lai CL (2006)
Characterization of fusion partner genes in 114 patients with de novo acute myeloid leukemia and MLL rearrangement.
Leukemia 20: 218-23



. AML with these fusions usually have myelomonocytic or monoblastic morphologic and immunophenotypic features. While in the past all of these translocations were encompassed by the category of AML with 11q23 abnormalities, the diagnosis should now specify the specific abnormality and should be limited to cases with 11q23 balanced translocations involving MLL. For example, a case of AML with an MLL-ENL fusion would be ?diagnosed as acute myeloid leukaemia with t(11;19)(q23;p13.3); MLL-ENL.

Acute myeloid leukaemias with cyto?genetic abnormalities associated with prior therapy or myelodysplasia, such as t(2;11) (p21;q23) or t(11;16)(q23;p13.3), should be diagnosed as therapy-related myeloid neoplasms or AML with myelodysplasia-related changes.

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