Table of Contents
Definition / general | Treatment | Microscopic (histologic) description | Positive stains | Negative stains | Molecular / cytogenetics description | Molecular / cytogenetics imagesCite this page: Mihova D. t(v;11q23); KMT2A rearranged. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/leukemiapreballMLL.html. Accessed November 28th, 2024.
Definition / general
- Rearrangements of MLL gene
- 20% of ALL overall (Anticancer Res 2005;25:1931) including 80% of infants (Leukemia 2007;21:633), 10% of older children and adults
- May have bilineal lymphoblasts with monoblasts and promonocytes
- Usually infants < 1 year with markedly increased WBC (> 100x109/L), CNS involvement; pure lymphomatous involvement not typical
- Poor prognosis (30% event free survival)
Treatment
- Intensive chemotherapy followed by hematopoietic stem cell transplantation
Microscopic (histologic) description
- No defining morphology
Positive stains
Negative stains
Molecular / cytogenetics description
- Over 75 genetic arrangements; most translocations at 11q23 involve MLL (mixed lineage leukemia) gene
- t(4;11)(q21;q23) - MLL-AF4: occurs in 60% of infants, 2% of other children and 3 - 6% of adults
- t(11;19)(q23;p13.3) - MLL-ENL and others
- MLL translocation is associated with FLT3 overexpression
- 11q23 deletions are NOT included in this group because different immunophenotype, clinical and prognostic features