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AP/CP Board Review Hemepath > Precursor Neoplasms: T and B-ALL/LBL > Flashcards

Precursor Neoplasms: T and B-ALL/LBL Flashcards

1
Q

What is the immunophenotype of

blasts of Precursor B-ALL/LBL, NOS?

A
  • Positive for:
    • CD19
    • CD10
    • PAX5
    • CD34
    • CD99, HLA-DR
    • Nuclear Tdt
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2
Q

How does the Burkitt Lymphoma

IHC panel compare to precursor B-ALL/LBL?

A
  • CD19 +
  • CD20 +
  • CD10 +
  • HLA-DR and CD99 +
  • TDT (-)
  • surface immunoglobulin (+)
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3
Q

What is the immunophenotype of

precursor T-ALL/LBL?

A
  • CD19 (-)
  • CD20 (-)
  • CD10 (-)
  • HLA-DR (-)
  • surface immunoglobulin (-)
  • CD7 and TdT (+)
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4
Q

What is the prognosis for patients with B-ALL/LBL

in children and adults?

A
  • Children: CR >95%
  • Adults: CR 80%

This is with therapy. Adults have a slightly worse prognosis.

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5
Q

What are the key prognostic variables for

B-ALL/LBL patients?

A
  • Favorable:
    • age: 1-9 years old
    • WBC: <50,000
    • good response to initial treatment
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6
Q

What genetic finding is associated with

a poor prognosis in B-ALL/LBL?

A
  • hypodiploidy (46 chromosomes)
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7
Q

What are general characteristics of

Lymphoblastic lymphoma and lymphoblastic leukemia?

A
  • LBL: lesions involve the tissue and spare the peripheral blood
  • ALL: lesions heavily involve the marrow and peripheral blood
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8
Q

When does ALL peak and

what are the characteristics of ALL?

A
  • peaks: at age 3
  • most childhood ALL = B-ALL
    • 10% are T-ALL
  • T-ALL is more common in males and teens
    • <15%
    • most are LBL 85-90%
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9
Q

What are risk factors for the development of

ALL/LBL?

A
  • Down Syndrome
  • Fanconi Anemia
  • Li-Fraumeni Syndrome
  • Ataxia-Telangiectasia
  • Bloom Syndrome
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10
Q

What is the characteristic clinical presentation

of T-LBL?

A
  • anterior mediastinal mass
  • may be associated with hypercalcemia
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11
Q

What is the characteristic clinical presentation of

B-LBL?

A
  • involves the lymph nodes, CNS, liver, spleen and testes
  • does not involve the mediastinum

Note: both present with fatigue and cytopenis with or without bone pain

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12
Q

What is the characteristic morphology of

T or B-ALL/LBL?

A
  • monotonous proliferation of undifferentiated blasts
  • T-LBL may be associated with infiltrating eosinophils
  • PAS-positive in block pattern
    • negative for MPO
    • negative for Sudan black B
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13
Q

What is the most common molecular finding

in B-ALL/LBL that has a poor prognosis?

A
  • t(9;22)
  • BCR-ABL
    • minor breakpoint 190 kd chimeric protein
    • different than the CML breakpoint which is 210
  • distinctly unfavorable finding
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14
Q

What are the translocation proteins, morphology, prognosis and age group of

B-ALL/LBL with t(9;22)?

A
  • genetics: BCR/ABL
  • age group:
    • adults: 25% (p210, p190)
    • children: 3% (p190)
  • poor prognosis
  • usual morphology
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15
Q

What are the IHC findings of

t(9;22) B-ALL/LBL?

A
  • CD19 +
  • CD10 +
  • Tdt +
  • CD34 +
  • CD13/33 + (weak)
  • CD25 +
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16
Q

What are the translocation proteins, morphology, prognosis and age group of

B-ALL/LBL with t(4;11)?

A
  • second translocation: t(v;11q23)
  • genetics: KMT2A (MLL)
  • seen in infants ( <1 year)
    • CNS involved
    • can start in utero
    • associated with Topoisomerase II inhibitors
  • poor prognosis
  • usual morphology
17
Q

What is the IHC of the t(4;11) B-ALL/LBL?

A
  • CD19 +
  • CD10 (-)
  • CD15 +
  • express FLT3
18
Q

What are the translocation proteins, morphology, prognosis and age group of

B-ALL/LBL with t(12;21)?

A
  • genetics: TEL-AML1 (ETV6-RUNX1)
  • 25% of children
  • good prognosis
  • usual morphology
19
Q

What is the IHC of B-ALL/LBL with

t(12;21) or TEL-AML1 (ETV6-RUNX1)?

A
  • CD10 +
  • CD19 +
  • CD9 (-)
  • CD13/33 + (weak)
  • CD34 +
20
Q

What are the translocation proteins, morphology, prognosis and age group of

B-ALL/LBL that are hyperdiploid ?

A
  • genetics: > 50 chromosomes
    • extra copies of esp 21, 4, 14, and X
  • seen in 25% of children
  • good prognosis
  • usual morphology
21
Q

What are the IHC findings for the

hyperdiploid B-ALL/LBL?

A
  • CD19 +
  • CD10 +
  • Tdt +
  • CD34 +
22
Q

What are the translocation proteins, morphology, prognosis and age group of

B-ALL/LBL with hypodiploid genetics ?

A
  • genetics: <46 chromosomes
  • < 5% of children and adults
  • poor prognosis
  • usual morphology
23
Q

What are the IHC findings for

B-ALL/LBL that are hypodiploid?

A
  • CD19 +
  • Tdt +
  • CD10 +
  • CD34 +
24
Q

What are the translocation proteins, morphology, prognosis and age group of

B-ALL/LBL with t(1;19)?

A
  • genetics: E2A-PBX1
  • pre-B-ALL phenotype
  • 5% of children
  • poor prognosis
  • usual morphology
25
Q

What are the IHC findings for

B-ALL/LBL with t(1;19)?

A
  • pre-B-ALL phenotype
  • CD10 +
  • CD19 +
  • CD34 (-)
  • CD9 (+)
26
Q

What are the translocation proteins, morphology, prognosis and age group of

B-ALL/LBL with t(5;14)?

A
  • genetics: IL3-IGH
  • seen in < 1% of children and adults
  • usual prognosis
  • associated with significant eosinophilia
    • not malignant eos
27
Q

What are the IHC findings for B-ALL/LBL

with t(5;14) (IL3-IGH)?

A
  • CD19 +
  • CD10 +
  • CD34 +
  • Tdt +
28
Q

What are the translocation proteins, morphology, prognosis and age group of

B-ALL/LBL with BCR-ABL like phenotype?

A
  • genetics: lack BCR-ABL translocation
    • similar expression profile
  • seen in high risk children, adults
  • poor prognosis
  • usual morphology
29
Q

What is the IHC profile seen in

BCR-ABL like B-ALL/LBL?

A
  • CD19 +
  • CD10 +
  • Tdt +
  • CD34 +
30
Q

What are the translocation proteins, morphology, prognosis and age group of

B-ALL/LBL with iAMP21 ?

A
  • genetics: RUNX1
  • seen in 2% of children
  • poor prognosis
  • usual morphology

IHC:

  • CD19 +
  • CD10 +
  • Tdt and CD34 +
31
Q

What is the typical presentation of

T-ALL-LBL?

A
  • commonly presents in adolescents
  • rapid growth with sparing of the bone marrow
  • most important is the initial response to treatment
  • difficult to distinguish from a thymoma in the anterior medistinum (refer to IHC)
32
Q

What are the IHC findings for

T-ALL/LBL?

A
  • Positive:
    • CD99, Tdt (cytoplasmic and nuclear)
    • CD2, CD3, CD5,
    • CD7
  • CD34 (variable)
  • HLA-DR negative
  • IMP: CD4 and CD8 are either both positive or both negative
  • TCR genes are rearranged, 20% have IGH arrangements
33
Q

What are the IHC findings of a thymoma to

help differentiate it from a T-LBL?

A
  • Thymic T cells coexpress: CD4 and CD8
  • Epithelial cells present in thymomas and stain with EMA
34
Q

What translocation and gene are seen in

T-LBL with eosinophilia and myeloid hyperplasia?

A
  • 8p11.2 syndrome
  • invovles the FGFR1 gene
35
Q

What are the criteria from the WHO to assign

Acute leukemia with mixed lineage?

A
  • must have following criteria to assign blast populatino > 1 lineage
  • Myeloid
    • MPO or monocytic differentiation (at least 2)
    • NSE, CD14, CD64, CD11b, Lysozyme
  • B lineage
    • need strong CD19 and 1 of the following:
      • CD79a, cytoplasmic CD22, and CD10
    • or weak CD19 and 2 of the following:
      • CD79a, cytoplasmic CD22, and CD10
  • T lineage
    • cytoplasmic CD3 by Flow
    • surface CD3 (rare)

AP/CP Board Review Hemepath (12 decks)

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