Non-Hodgkin Lymphomas

Question 1

What is the clinical staging of lymphomas?

Answer 1

Ann Arbor Classification

Stage I: single LN region (I) or single extralymphatic site (IE)

Stage II: two or more LN regions on same side of diaphragm (II) or localized extralymphatic site (IIE)

Stage III: LN regions on both sides of diaphragm (III) w/ or w/o localized extralymphatic site (IIIE)

Stage IV: multiple, diffuse extralymphatic sites w/ or w/o lymphatic involvement

Question 2

What is the most common and most deadly plasma cell cancer?

Answer 2

multiple myeloma

Question 3

What is follicular lymphoma? (cell type and key features)

Answer 3

• germinal center B cell lymphoma (non-Hodgkin)
• t(14:18) involivng Ig heavy chain and antiapoptotic BCL2 (normally absent in germinal centers)
• nodular masses in lymphnodes
• normal B cell markers

Question 4

What is the common presentation of follicular lymphoma?

Answer 4

• middle aged adults
• painless, generalized lymphadenopathy

Question 5

What are potential complications of follicular lymphoma?

Answer 5

-transformation to large B-cell lymphoma

Question 6

What is the course/prognosis of CLL/SLL?

Answer 6

• indolent course but uncurable
• survival median of 7-9 years
• palliative chemotherapy

Question 7

What is the most common form of non-Hodgkin lymphoma?

Answer 7

diffuse large B cell lymphoma

Question 8

What is diffuse large B cell lymphoma? (cell type and key features)

Answer 8

• large (4-5x normal) mature B cells
• non-Hodgkin lymphoma

-normal B cell markers

-occurs spontaneously or from transformation of other cancers

Question 9

What is the common presentation of diffuse large B cell lymphoma?

Answer 9

• occurs in all ages; more common in adults (median age 60)
• rapidly enlarging lymph node

Question 10

What is the course/prognosis of diffuse large B cell lymphoma?

Answer 10

aggressive course

Prognosis:

• rapidly fatal without treatment
• remission: 60-80%
• cure: 40-50%

Question 11

What is Burkitt lymphoma? (cell type and key features)

Answer 11

• germinal B cell
• associated with EBV infection
• t(8:14) involivng oncogene c-myc and Ig heavy chain
• “starry sky” appearance (sky = cancer cells, stars = occasional macrophage phagocytizing apoptotic cancer cells)
• normal B cell markers

Question 12

What are the different presentations of Burkitt lymphoma?

Answer 12

Common to both:

• child/young adult
• extranodal mass

African:

-lesion on jaw

Sporadic:

-lesion in abdomen

Question 13

What is the course/prognosis of Burkitt lymphoma?

Answer 13

Aggressive course

Prognosis:

-most younger individuals cured; worsens with older age1

Question 14

What is lymphoplasmacytic lymphoma?

Answer 14

Also called Waldenström macroglobulinemia

• plasma cell cancer
• excess production of monoclonal IgM -> serum hyperviscosity

-MyD88 association

Question 15

What is the common presentation of lymphoplasmacytic lymphoma?

Answer 15

• nonspecific
• lymphadenopathy
• anemia
• *vision problems
• *neurologic effects (HA,, dizziness, stupor)
• *bleeding
• *cryoglobulinemia (Raynaud phenomenon)

*associated with increased serum viscosity/high IgM

Question 16

What is the course/prognosis of lymphoplamacytic lymphoma?

Answer 16

Indolent course

plasmaphoresis to remove IgM improves symptoms

incurable, meidan survivial is 4 years

Question 17

Compare lymphblastocytic lymphoma to CLL and MM.

Answer 17

CLL:

• similar in presentation (asymptomatic, present in LNs, )
• CLL does not have hyperviscosity sydrome

MM:

• MM produces IgG/A instead of bulkier IgM, hence no hyperviscosity in MM
• MM has bone lesions

Question 18

What is mantle cell lymphoma?

Answer 18

• naive B cell lymphoma
• t(11;14) involving cyclin-D1 and IgH promoting G1/S transition
• CD5⁺, CD23^-, CD19, CD20 (CD23^- distinguished from CLL)

Question 19

What is the course/prognosis of mantle cell lymphoma?

Answer 19

moderately aggressive course

poor prognosis; median survival 3-4 years

no cure

Question 20

What is the common presentation of mantle cell lymphoma?

Answer 20

• Older men
• painless lymphadenopathy

Question 21

What is marginal zone lymphoma?

Answer 21

• heterogenous B cell lymphoma
• extranodal sites in chronic inflammatorys states (salivary glands in Sjögrens, thyroid in Hashimoto thyroiditis, and stomach with H. pylori infection

Question 22

What is the course/prognosis of marginal zone lymphomas?

Answer 22

early stages can be reveresed by removing chronic inflammatory state (ie. treating H. pylori)

Later stages, tumor have mutations that allow them to survive without the chronic inflammatory state

Question 23

What is extranodal NK/T-cell lymphoma?

Answer 23

• cancer of NK cells
• CD3^-, TCR^-, CD16⁺, CD56⁺
• EBV infection

Question 24

What is the common presentation of extranodal NK/T-cell lymphoma?

Answer 24

• adults
• destructive extranodal masses, most commonly nasopharyngeal

Question 25

What is the course/prognosis of extranodal NK/T-cell lymphoma?

Answer 25

aggressive course

poor prognosis

Question 26

What is anaplastic large-cell lymphoma?

Answer 26

• cancer of CD8 T cells
• ALK⁺ (not normally present in lymphocytes)

-CD30+ (also found on Reed-Sternberg cells)

• large “hallmark” cells with horseshoe nuclei
• cluster around venules/lymphoid sinuses mimicing metastatic carinoma

Question 27

What is the common presentation of anaplastic large-cell lymphoma?

Answer 27

-children and young adults (unusual for T cell neoplasms)

-lymphadenopathy

Question 28

What is the course/prognosis of anaplastic large-cell lymphoma?

Answer 28

aggressive course

very good prognosis; 75-80% cure (unusual for T cell neoplasms)