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Disease and Therapeutics 1 > Precursor B and T Cell Neoplasms and Mature B Cell Neoplasms > Flashcards

Precursor B and T Cell Neoplasms and Mature B Cell Neoplasms Flashcards

1
Q

PreT ALL is usually…

A

lymphomic

ex- you may see a mediastinal mass

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2
Q

Epidemiology of Precursor B and T Cell Neoplasms: how common? Race? Gender? Age for each?

A

Precursor B and T Cell Neoplasms are the most common cancer of children; 5/100,000 age 2-5
Hispanics > Whites >&raquo_space; Blacks
Boys > Girls
Pre B incidence is highest at 4yrs; Pre T incidence is highest at adolescence

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3
Q

Describe the morphology of Precursor B and T Cell Neoplasms

A
  • Both look similar
  • Small cells, high N/C Ratio, irregular nuclear contours, immature nuclear chromatin, +/- nucleoli (look similar to a “hand mirror”)
  • No peroxidase granules (MPO negative)
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4
Q

Immunophenotype of immature lymphocytes

A

CD34 and TdT positive; surface light chain negative

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5
Q

Immunophenotype of Pre-B Cells

A

CD19, CD22, CD10

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6
Q

Immmunophenotype of Pre-T Cells

A

Early cell- CDIa, CD2, CD5, CD7

Other T cell antigens- cCD3, CD4/8

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7
Q

Genetics of Pre-B Neoplasms

A
  • Loss of fxn mutations
  • translocation 12,22 involving ETV6 and RUNXI genes
  • translocation 9,22 involving BCR and ABL genes
  • Hyperdiploidy or Hypodiploidy
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8
Q

Genetics of Pre-T Neoplasms

A

NOTCHI mutations (gain of fxn)

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9
Q

Favorable prognostic factors for Precursor B and T Cell Neoplasms include

A 
Age 2-10
Low WBC count
Early Pre-B Phenotype (CD19/CD10)
Hyperdiploidy
Trisomy of 4, 7, 10
12,21 translocation
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10
Q

Poor prognostic risk factors for Precursor B and T cell neoplasms

A

Less than 2yrs old
MLL gene on chromosome 11
Adult
Peripheral Blast count > 100,000

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11
Q

Clinical Features of Chronic Lymphocytic Leukemia (CLL)/ Small Lymphocytic Lymphoma (SLL) (5)

A
  1. Patient older than 50
  2. Typically asymptomatic
  3. Adenopathy, hepatosplemomegaly
  4. May have monoclonal serum spike
  5. Immune disruption
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12
Q

Morphology of CLL

A

Peripheral Blood- increase in small mature lymphocytes with hyperclumped nuclear chromatin; smudge cells

BM- Lymphoid aggregates
Spleen/Liver- red and white pulp; portal tracts

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13
Q

Morphology of SLL

A

Lymph nodes effaced diffusely by small round cells, proliferation centers mimic germinal centers

BM- lymphoid aggregates
Spleen/Liver- red and white pulp; portal tracts

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14
Q

In CLL/SLL, what are the pale regions observed in the lymph nodes that have replaced the follicles?

A

Proliferation centers that are sites of neoplastic proliferation and diffuse replacement of normal tissue by small lymphocytes

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15
Q

Pre-B ALL is usually…

A

Leukemic

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16
Q

Immunophenotype of CLL/SLL

A

CD19, CD5, CD23 and dim CD20 and dim light chain restricted

CLL dimly express kappa and not lambda

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17
Q

Genetics of CLL/SLL

A

Deletions: 13q14, 11q and 17p
Trisomy 12

Severity (Good –>Bad) 13q14, tris12, 11q, 17p

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18
Q

What is Richter Syndrome?

A

The transformation of CLL to Diffuse Large B Cell Leukemia (which has a poor prognosis and a higher grade)

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19
Q

Follicular Lymphoma is associated with what translocation?

A

Translocations involving BCL2

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20
Q

Morphology of Follicular Lymphoma

A

Nodular pattern in lymph nodes
Centrocytes and Centroblasts
Paratubular aggregates seen in bone marrow
Portal tracts in the white pulp of the spleen and liver

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21
Q

How can you distinguish follicular lymphoma from follicular hyperplasia?

A

Follicular Lymphoma has no tingable macrophages and the nodules are basically taking over the lymph node

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22
Q

Immunophenotype of Follicular Lymphoma

A

CD19, CD20, CD10, BCL2 positive

bcl6

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23
Q

Genetics of Follicular Lymphoma

A

translocation of 14,18

14 = IgH
18 = BCL2
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24
Q

If Follicular Lymphoma transforms it becomes what higher grade cancer?

A

Diffuse Large B Cell Leukemia

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25
Q

Describe the morphology of Mantle Cell Lymphoma

A

Can have nodular or diffuse pattern
small round, slightly irregular lymphocytes
Surround and infiltrate normal germinal center
Cells are small with cleaved nuclear contours similar to SLL but expres cyclin D
Blastoid variant is more aggressive

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26
Q

Immunophenotype of Mantle Cell Lymphoma

A

CD19, CD20, CD5, bright surface light chain

CD23 negative

27
Q

Genetics of Mantle Cell Lymphoma

A

11, 14 translocation

11= cyclin D; 14 = IgH

28
Q

What are the treatment options for Mantle Cell Lymphoma?

A
  1. Chemotherapy + Retuximab (anti-CD20)

2. BM transplant

29
Q

What type of cancer arises from chronic inflammatory disorders?

A

Marginal Zone Lymphoma

30
Q

Describe the morphology of Marginal Zone Lymphoma

A

-Monocytoid B cells and plasmactoid cells that infiltrate and destroy host tissue

31
Q

Immunophenotype of Marginal Zone Lymphoma

A

CD20, CD19 and surface light chain restricted

32
Q

Genetics of Marginal Zone Lymphoma

A

Inflammation –> acquisition of mutations
11,18 transposition
1,14 transposition
Upregulation of BCL10 and MALT1

33
Q

What cell population is largely neoplastic in lymphoplasmacytic lymphoma?

A

Plasma Cells

34
Q

Secretion of IgM from Lymphoplasmacytic Lymphomas can cause what?

A

A hyperviscosity syndrome called Waldenstrom Macroglobulinemia

35
Q

What are the symptoms of Waldenstrom’s Macroglobulinemia from LPL?

A
  1. Visual Symptoms
  2. Neurologic Symptoms
  3. Bleeding (because it interferes with the coagulation factors and platelets)
  4. Cryoglobulinemia (IgM that precipitates at low temps in the fingers/toes)
36
Q

A mutation in what gene is associated with LPL?

A

MYD88

37
Q

BRAF mutation is associated with what?

A

Hairy Cell Leukemia

38
Q

What are the clinical symptoms of Hairy Cell Leukemia?

A

Splenomegaly and Pancytopenia

39
Q

Describe the morphology of Hairy Cell Leukemia

A

Diffuse, fried egg appearance in Bone Marrow
Round hyperchromatic nuclei with pale blue cytoplasm which have hairs, making the borders indistinct
Cells incite reticulin fibrosis on dry BM tap
Causes obliteration of the white pulp and involves red pulp

40
Q

Immunophenotype of Hairy Cell Leukemia

A

CD19, CD20, surface light chain restriction

CD11c/CD25/CD103

41
Q

Treatment of Hairy Cell Leukemia

A

Use a purine analog chemo –> long remission

BRAF inhibitors

42
Q

What is secreted by all plasma cell neoplasms?

A

M Component

Bruce Jones Protein (free light chain)

43
Q

Immunophenotype of Plasma Cell Neoplasms

A

CD38, CD56

No- CD20, CD19 and CD45

44
Q

The weakness and fatigue of MM is caused by

A

Anemia

45
Q

The recurrent infections seen with MM are caused by

A

Decreased IgG

46
Q

The polyuria, constipation and confusion seen with MM is caused by

A

Hyercalcemia

47
Q

The bone pain and fractures associated with MM are caused by

A

the tumor load

48
Q

Renal Insufficiency seen in MM is caused by

A

Bence Jones Protein

49
Q

Amyloidosis seen in MM is caused by

A

Light chain deposition in organs

50
Q

Radiographic studies of Multiple Myeloma will reveal

A

Bony Lytic Lesions (vertebral column, ribs, skull and pelvis)

51
Q

Multiple Myeloma is associated with what genetic changes?

A

translocations involving IgH, deletions of 17p (TP53) and rearrangements involving MYC

Tumor Produces IL6

52
Q

In MM, the increased serum immunoglobulins cause

A

RBC adhesion resulting in “rouleaux formation”

53
Q

What are the common clinical features of Diffuse Large B Cell Lymphome (DLBCL)?

A

Rapidly enlargine lymph node or extranodal site; possible B type symptoms

54
Q

Describe the morphology of DLBCL

A

Large lymphocytes with convoluted nuclear contours, 1-3 nucleoli, pale cytoplasm and indistinct cellular borders (mitotically active)

55
Q

Immunophenotype of DLBCL

A

CD19, CD20, surface light chain restriction

+/- bcl2, CD10, BCL6

56
Q

Genetics of DLBCL

A
  • BCL6 (3q27) mutation –> represses normal B cell differentiation, growth arrest and apoptosis
  • BCL2 (18) mutation
  • MYC (8) mutation or translocation (patients with the translocation do better than those with the mutation)
57
Q

Which mature B cell neoplasm has the highest grade?

A

Burkitt Lymphoma

58
Q

Burkitt Lyphoma is strongly associated to what virus?

A

EBV

59
Q

What are the clinical features of Burkitt Lymphoma?

A

Tumor Lysis Syndrome and CNS diseases

60
Q

What is Tumor Lysis Syndrome?

A

Rapid cell turnover resulting in tumor cell death and the release of uric acid, K and Ca –> medical emergency requiring hydration, binding agents for electrolytes and hemodialysis

Occurs in Burkitt Lymphoma

61
Q

Describe the morphology of Burkitt Lymphoma

A

Diffuse infiltrate of medium size cells with round nuclear contours, basophilic cytoplasm and vacuoles, proliferation rate at 100% (measure by Ki-67) and many macrophages giving the appearance of a starry sky

62
Q

Why does Burkitt Lymphoma grow so fast?

A

MYC translocation –> increased exp of genes for glycolysis so constantly making building blocks for growth and division

63
Q

Immunophenotype of Burkitt Lymphoma

A

CD19, CD20, CD10, surface light chain restriction, bcl6

No bcl2, CD34 or TdT

64
Q

The translocations involving MYC seen in Burkitt Lymphoma include

A

8, 14
2, 8
8, 22

Disease and Therapeutics 1 (7 decks)

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