Bone marrow neoplastic

Bone marrow - plasma cell and lymphoid neoplasms

B lymphoblastic leukemia / lymphoma with recurrent genetic abnormalities

t(v;11q23); KMT2A rearranged



Last author update: 1 February 2013
Last staff update: 18 March 2022

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PubMed Search: B lymphoblastic leukemia MLL rearranged

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Cite this page: Mihova D. t(v;11q23); KMT2A rearranged. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/leukemiapreballMLL.html. Accessed March 19th, 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
Molecular / cytogenetics images

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ALL1 / MLL duplication

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