Leukemia - Acute
PreB ALL
B cell acute lymphoblastic leukemia (ALL) / lymphoblastic lymphoma (LBL)

Author: Daniela Mihova, M.D. (see Authors page)

Revised: 11 April 2018, last major update March 2013

Copyright: (c) 2001-2018, PathologyOutlines.com, Inc.

PubMed Search: B cell acute lymphoblastic leukemia ALL lymphoblastic lymphoma LBL

Cite this page: Mihova, D. B cell acute lymphoblastic leukemia (ALL) / lymphoblastic lymphoma (LBL). PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/leukemiapreBall.html. Accessed August 16th, 2018.
Definition / general
  • Current 2008 WHO classification: B lymphoblastic leukemia / lymphoma, NOS or B lymphoblastic leukemia / lymphoma with recurrent genetic abnormalities
  • See also lymphomas: B cell chapter
  • Also called B cell acute lymphoblastic leukemia / lymphoblastic lymphoma, pre B ALL / LBL
  • Usually children
  • B acute lymphoblastic leukemia presents with pancytopenia due to extensive marrow involvement, stormy onset of symptoms, bone pain due to marrow expansion, hepatosplenomegaly due to neoplastic infiltration, CNS symptoms due to meningeal spread and testicular involvement
  • B acute lymphoblastic lymphoma often presents with cutaneous nodules, bone or nodal involvement, < 25% lymphoblasts in bone marrow and peripheral blood; aleukemic cases are usually asymptomatic
  • Depending on specific leukemia, arises in either hematopoietic stem cell or B-cell progenitor
  • Tumors are derived from pre-germinal center naive B cells with unmutated VH region genes
  • Have multiple immunophenotyping aberrancies relative to normal B cell precursors (hematogones); at relapse, 73% show loss of 1+ aberrance and 60% show new aberrancies (Am J Clin Pathol 2007;127:39)
Prognostic factors
  • Favorable prognosis: age 1 - 10 years, female, white; preB phenotype, hyperdiploidy > 50, t(12,21), WBC count at presentation < 50x108/L, non-traumatic tap with no blasts in CNS, rapid response to chemotherapy < 5% blasts on morphology on day 15, remission status after induction < 5% blasts on morphology and < 0.01% blast on flow or PCR, CD10+
  • Intermediate prognosis: hyperdiploidy 47 - 50, diploid, 6q- and rearrangements of 8q24
  • Unfavorable prognosis: under age 1 (usually have 11q23 translocations) or over age 10; t(9;22) (but not if age 59+ years, Am J Clin Pathol 2002;117:716); male, > 50x108/L WBC at presentation, hypodiploidy, near tetraploidy, 17p- and MLL rearrangements t(v;11q23); CD10-; non-traumatic tap with > 5% blasts or traumatic tap (7%); also increased microvessel staining using CD105 in children (Leuk Res 2007;31:1741), MDR1 expression in children (Oncol Rep 2004;12:1201) and adults (Blood 2002;100:974), 25%+ blasts on morphology on day 15, remission status after induction ≥ 5% blasts on morphology and ≥ 0.1% blasts on flow or PCR
Case reports
Treatment
Microscopic (histologic) description
  • Bone marrow smears: small to intermediate blast-like cells with scant, variably basophilic cytoplasm, round / oval or convoluted nuclei, fine chromatin and indistinct nucleoli; frequent mitotic figures; may have "starry sky" appearance similar to Burkitt lymphoma; may have large lymphoblasts with 1 - 4 prominent nucleoli resembling myeloblasts; usually no sclerosis
  • Bone marrow biopsy: usually markedly hypercellular with reduction of trilinear maturation; cells have minimal cytoplasm, medium sized nuclei that are often convoluted, moderately dense chromatin and indistinct nucleoli, brisk mitotic activity
  • Other tissues: may have "starry sky" appearance similar to Burkitt lymphoma; collagen dissection, periadipocyte growth pattern and single cell linear filing
Microscopic (histologic) images

Images hosted on PathOut server:

Peripheral blood:
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L1 type has smaller blasts with minimal
cytoplasm, coarse chromatin, some
cleaved nuclei or irregular contours
and no distinct nucleoli



Images hosted on other servers:

Peripheral blood:
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Blasts have L1 morphology,
but mature phenotype



Lymphoblastic lymphoma:
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TdT negative cases



Lymphoblastic lymphoma with basal cell carcinoma:
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TdT+

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CD79a+

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CD34+



Kidney involvement
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19 year old man with hypertension
and renal insufficiency

Positive stains
Negative stains
Flow cytometry Images

Images hosted on other servers:
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Patient with relapse of precursor B-ALL
illustrating multiple antigenic aberrancies

Molecular / cytogenetics description
  • Usually chromosomal abnormalities
Differential diagnosis