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Leukemia - Acute

PreB ALL

B lymphoblastic leukemia / lymphoma with t(v;11q23); MLL rearranged


Reviewer: Daniela Mihova, M.D. (see Reviewers page)
Revised: 2 February 2013, last major update September 2012
Copyright: (c) 2001-2013, PathologyOutlines.com, Inc.

General
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● 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
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● Intensive chemotherapy followed by hematopoietic stem cell transplantation

Micro description
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● No defining morphology

Positive stains
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● TdT, CD19, CD34 and HLA-DR
● Also CD15, often myeloid antigens CD13 and CD33 (Am J Clin Pathol 1999;111:467)

Negative stains
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● CD10

Molecular description
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● 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 images
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Diagram of ALL1 / MLL duplication

End of Leukemia - Acute > PreB ALL > B lymphoblastic leukemia / lymphoma with t(v;11q23); MLL rearranged


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