Lymphomas Flashcards

1
Q

List 3 important causes of lymphadenopathy:

A

Reactive (benign) - e.g in response to immune system, cell type is stimulated and architecture is preserved

Lymphoma (either Hodgkin or Non-hodgkin)

Metastatic disease

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2
Q

Follicular hyperplasia:

A

Proliferation of B lymphocytes

Increased need for antibody production

Enlargement of germinal centers, with tingible-body macrophages and increased mitotic activity

E.g. enlarged cervical nodes in strep throat

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3
Q

Paracortical hyperplasia:

A

Proliferation of T lymphocytes

Increased need for cell-mediated immunity

Expansion of paracortical regions, with increased mitotic activity and activated lymphocytes

E.g. neck nodes in patient with infectious mono

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4
Q

Sinus histiocytosis:

A

Proliferation of histiocytes (aka tissue macrophages)

Stimulation of antigen-presenting cells

expansion of subcapsular/medullary sinuses

e.g. lymph nodes draining a carcinoma

Classically see this in patients with cancer

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5
Q

3 benign reactive causes of lymph node enlargement:

A

Follicular pattern (B cell proliferation)

Paracortical pattern (T cell proliferation)

Sinus histiocytosis (histiocytic proliferation)

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6
Q

Non-Hodgkin Lymphoma incidence and risk factors:

A

Highest in US, Australia, and Europe

Age

Infections

Immune disorders

Toxins

However, while risk factors are important, most patients have no identiafiable risk factor

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7
Q

How does the WHO classify lymphomas?

A

Based on the cell of origin.

E.g. breaks down into Hodgkin vs. Non-Hodgkin, then into B cell and T cell, further by e.g. precursor B cell neoplasms and Mature B cell neoplasms.

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8
Q

How does The Working Formulation classify lymphomas?

A

Low grade - can live years without treatment

  • Small lymphoblastic lymphoma
  • Follicular small cleaved lymphoma

Intermediate grade - can live a year

  • Diffuse large cell lymphoma

High grade - months of survival

  • Burkitt lymphoma
  • Lymphoblastic lymphoma
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9
Q

______ is associated with an abnormal growth pattern in lymph node.

A

Lymphoma

Loss of architecture

(absence of germinal centers and sinuses)

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10
Q

What disease processes are indicated in these photos?

A

Left - Follicular Lymphpma (node contains dozens of enlarged follicles, caused by overexpression of BCL2)

Right - diffuse, characteristic of e.g. Diffuse Large B cell lymphoma, which can arise sporadically or from Follicular Lymphoma

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11
Q

SLL and CLL both:

A

Comprised of small, round lymphocytes

Occur in older adults

Have the same immunophenotype

Indolent but considered incurable

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12
Q

Explain the markers expressed on B cells in SLL:

A

Mature B cells, so:

CD19

CD20

CD22

CD23

BUT co-express CD5

Kappa OR lamda light chains

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13
Q

____ is associated with a good SLL prognosis.

____ is associated with a poor SLL prognosis.

A

13q14 is associated with a good SLL prognosis.

trisomy 12 is associated with a poor SLL prognosis.

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14
Q

What disease is suggested by this peripheral smear?

A

Could be CLL (chronic lymphocytic leukemia) or peripheral blood involvement by SLL (small lymphocytic lymphoma)

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15
Q

Follicular Lymphoma is commonly found in the ___ population. Characterized by WHAT mutation?

A

Found in adults.

Characterized by t(14;18) - this results in a BCL2 proto-oncogene translocated downstream of IgH gene regulatory sequences

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16
Q

Follicular Lymphoma causes an abnormal immunophenotype of _____ cells

A

Mature B cells

(cells resemble normal germinal center cells)

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17
Q

How is Follicular lymphoma treated? How can it progress if uncreated?

A

Indolent course - median survival 10 years

Incurable with conventional chemo

Patients typically present with high stage disease (including marrow involvement)

May progress to diffuse large cell lymphoma

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18
Q

What markers are present on B cells in patients with Follicular lymphoma?

A

CD19

CD20

CD22

CD10

Kappa OR lambda light chain

19
Q

What disease is indicated by this bone marrow biopsy?

A

Mantle Cell Lymphoma

See numerous small lymphocytes with mixed in pink histiocytes (tissue macrophages).

20
Q

What CD markers are present in patients with Mantle Cell Lymphoma?

A

Mature B cells positive for:

CD5

CD19

LAMBDA chain (almost always)

NEGATIVE FOR CD10 and CD23

21
Q

Mantle Cell Lymphoma is associated with what diagnostic gene arrangement? What cellular processes result from this?

A

t(11;14)

Cyclin D1 gene from 11 is moved to IgG gene location on chromosome 14, resulting in high expression of cyclin D1.

Cyclin D1 promotes G1 –> S phase of cell cycle

22
Q

Diffuse Large B-Cell Lymphoma occurs in what patient population?

A

All ages - children and adults

Can be associated with immune dysfunction (e.g. HIV, SCID, BM or organ transplants)

23
Q

Diffuse Large B cell Lymphoma is often associated with an abnormal _____.

A

BCL-6

24
Q

Can Diffuse B cell lymphoma be cured?

A

Yes - low stage disease can be cured

25
Q

Approximately ____ of cases of Diffuse B cell Lymphoma are _____.

A

Extranodal

Occur in places like the GI tract and Brain

26
Q

In what populations is Burkitt’s Lymphoma found?

A

Predominantly in children

Endemic (Africa; >95% EBV positive)

Non-endemic (worldwide; 15-20% EBV positive)

27
Q

What are the cytogenetic characteristics of Burkitt’s Lymphoma?

A

t(8;14) is the most common

Also see t(2;8) and t(8;22)

c-myc (chrom 8) proto-oncogene downstream of IgH gene

28
Q

Burkitt Lymphoma is closely associated with ____

A

ALL FAB L3

29
Q

Burkitt Lymphoma is characterized by a mature _____ cell phenotype

A

Mature B-cell

30
Q

What disease is indicated by this bone marrow biopsy?

A

Burkitt’s Lymphoma

See tons of intermediate/large cells and spots of “starry sky” (tingible body macrophages).

These macrophages are gobbling up cell garbage.

31
Q

What is indicated by this biopsy?

A

Burkitt’s Lymphoma

See characteristic cytoplasmic vacuoles

32
Q

What leukemia is related to Lymphoblastic Lymphoma?

A

ALL - This is the lymph node correlate of ALL. BOTH:

  • are diseases of children
  • consist of lymphoblasts with similar phenotypes
  • are treated similarly
33
Q

Lymphoblastic lymphoma is a disease commonly found in ____ (like ALL). However, unlike ALL, this is more likely to be found in ____ and present with _____.

A

Lymphoblastic lymphoma is a disease commonly found in children (like ALL). However, unlike ALL, this is more likely to be found in teenagers and present with mediastinal mass.

34
Q

What is the most common immunophenotype associated with Lymphoblastic Lymphoma?

A

An immature T cell expressing CD-3, co-expressing CD4 & CD8 and TdT.

35
Q

An immature T cell expressing CD-3, co-expressing CD4 & CD8 and TdT is characteristic of what disease?

A

Lymphoblastic Lymphoma

36
Q

Hodgkin Lymphoma is characterized by a distinctive cell called the:

A

Reed-Sternberg Cell

37
Q

What is a Reed-Sternberg cell?

A

An activated B cell found in Hodgkin’s Lymphoma

38
Q

What population is Hodgkin’s found in?

A

Peak incidence in 20s and again in >50s

EBV present in about 40% of cases

39
Q

What is the common symptomatic presentation of Hodgkin’s Lymphoma? Why?

A

Commonly presents with enlarged lymph node - spreads along contiguous or adjacent lymph nodes (extranodal involvement is unusual)

40
Q

How is Hodgkin’s diagnosed? What CD markers, smear findings, etc. are associated with it?

A

Diagnoses based on finding Reed-Sternberg cells in the appropriate cellular background of:

Lymphocytes, eosinophils, histiocytes, fibroblasts

CD30+, CD15+, and CD45-

(neither flow cytometry nor cytogenetics are helpful because there aren’t many malignant cells)

41
Q

What morphologic subtypes are associated with Hodgkin?

A

Nodular sclerosis

Lymphocyte rich

Mixed cellularity

Lymphocyte depletion

42
Q

What is the likely diagnosis of this patient?

A

Hodgkin Lymphoma - Nodular Sclerosis Subtype

43
Q

What is the likely diagnosis of this patient?

A

Hodgkin Lymphoma

These arrows point to a Reed-Sternberg cell (characteristic “owl eyes” - large cell with nucleoli)

44
Q

What lymphoma is characteristically CD15 and CD30 positive, but CD45 negative?

A

Hodgkin Lymphpma