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DT 1 Test 2 > Precursor B and T cell Neoplasms > Flashcards

Precursor B and T cell Neoplasms Flashcards

1
Q

What is Acute Lymphoblastic Leukemia/Lymphoma (ALL)?

A

Neoplastic population of immature lymphocytes - lymphoblasts

Most ALL’s are pre-B - usually leukemia

Pre T ALL usually lymphomic - mediastinal mass

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2
Q

What is the mos common cancer of children?

A

ALL

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3
Q

What are the demographics associated with ALL?

A

Hispanic > White > Black

Boys > Girls

Children > adults

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4
Q

What are the clinical features of ALL?

A

Neoplastic cells crowd out normal marrow cells - marrow failure

Bone Pain

Generalized adenopathy, hepatosplenomaegaly

CNS sx

Pre-T associated with airway compression

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5
Q

What is the phenotype of of ALL cells?

A

CD34 and TdT positive

CD45 negative

MPO negative

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6
Q

What antigens are present on Pre-B ALL cells?

A

CD19

CD22

CD10

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7
Q

What antigens are present on Pre-T ALL cells?

A

CD2, cCD3, CD5, CD7

Possibly CD4/8

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8
Q

What translocations and genes are associated with B-ALL?

A

t(12;21) - ETV6 and RUNX1 genes - disturb differentiation and maturation

t(9;22) - BCR and ABL1 genes - tyrosine kinase activity

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9
Q

What mutation is associated with T-ALL?

A

NOTCH1 gene mutation - essential for normal T cell development

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10
Q

What is the treatment/prognosis of ALL?

A

Aggressive chemo plus CNS prophylaxis

Children - complete remission >95%

Adults - 35-40% cure

Molecular detection after therapy is associated with worse outcome

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11
Q

What are favorable prognostic factors for ALL?

A

Low WBC

Hyperdiploidy

Trisomies 4, 7, 10

t(12;21)

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12
Q

What are poor prognostic factors for ALL?

A

t(9;22) in Pre-B ALL

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13
Q

What is the most common leukemia of adults in the western world?

A

Chronic lymphocytic leukemia

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14
Q

What are the clinical features of CLL/SLL?

A

Patients > 50 years

Nonspecific generalized adenopathy, hepatosplenomegaly

Immune disruption - infections = hypogammaglobulinemia

Hemolytic anemia or thromobytopenia due to autoantibodies created by non-neoplastic B-cells due to immune dysregulation

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15
Q

What is the morphology of CLL on a peripheral blood smear?

A

Small mature lymphs with hyperclumped nuclear chromatin; smudge cells

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16
Q

What is the immunophenotype of CLL/SLL?

A

CD19/CD5/CD23

CD20 - dim

Surface light chain restricted - dim

17
Q

What is Richter syndrome?

A

Transformation of CLL/SLL to Diffuse Large B cell lymphoma

Rapidly enlarging lymph node and/or spleen

Most patients survive <1 year

18
Q

What is follicular lymphoma?

A

Second most common NHL

Strongly associated with translocations involving BCL2

19
Q

What are the clinical features of follicular lymphoma?

A

Painless adenopathy, generalized or localized

BM almost always involved - stage 4

20
Q

What is the histologic pattern of lymph nodes in follicular lymphoma?

A

Nodular or nodular and diffuse pattern

Centrocytes and centroblasts

21
Q

What proteins are present in follicular lymphoma and can be used as diagnosis?

A

BCL2 and BCL6 expression

22
Q

What translocation is associated with follicular lymphoma?

A

t(14;18)

14- IGH

18- BCL2

23
Q

What is the prognosis of follicular lymphoma?

A

Incurable, but indolent

24
Q

What do mantle cell lymphoma cells overexpress?

A

Aberrantly express CD5

Overexpression of Cyclin D1

25
Q

What are the clinical features of mantle cell lymphoma?

A

Painless, generalized lymphadenopathy

BM involvement

Lymphomatoid Polyposis = GI involvement

26
Q

What is the immunophenotype of mantle cell lymphoma?

A

CD19/CD20/CD5

Bright surface light chain

27
Q

What is the prognosis of mantle cell lymphoma?

A

Not curable, 3-5 years

Most patients die of organ failure due to tumor infiltration

DT 1 Test 2 (8 decks)

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