Heme Malignancy 3: CLL and B cell lymphomas

Question 1

What is the best diagnosis in the patient with the above peripheral smear? The cell population identified in the slide (arrow) comprises 60% of the peripheral blood leukocytes. There is an Auer rod in one cell.

A.Acute myeloid leukemia

B.Chronic myeloid leukemia

C.Acute lymphoblastic leukemia

D.Primary myelofibrosis

E. Essential thrombocythemia

Answer 1

A.

Question 2

What is the most likely finding in the patient with the above peripheral smear? The cell population denoted in the second slide comprises 60% of the peripheral blood leukocytes

A.Positive for T(15;17)

B.No translocations

C.Positive for JAK2 mutation

D.Positive for t(9;22) translocation

E.Positive for NOTCH1 mutation

Answer 2

A.

Question 3

What is the most likely diagnosis in the 15 yr old patient with a mediastinal mass and the above peripheral smear? Based on flow studies, the cells express CD2, CD3, CD7 and TdT

A.Chronic lymphocytic leukemia

B.T-acute lymphoblastic lymphoma/leukemia

C.B-acute lymphoblastic lymphoma/leukemia

D.Acute myeloid leukemia

Answer 3

B. (remember a T-ALL teenager with a thymic mass)

Question 4

Label the following on the lymph node below:

Germinal center

Mantle zone

Secondary follicle

Paracortex

Answer 4

see image below

Question 5

Label the CD markers in the regions marked below:

Answer 5

see below

**

CD10 is a specific germinal center marker

B cells in the germinal center are negative for Bcl2 (Bcl2 is expressed in cells everywhere else in the lymph node). Bcl2 is an anti-apoptotic protein so in the event that the B cells start to go awry, you would want them to die which is why Bcl2 is downregulated in the germinal center

Question 6

Hodgkin’s lymphoma arises from B cells in the ___ of the lymph node and can be subdivided into ___ and ___ types

Answer 6

Hodgkin’s lymphoma arises from B cells in the germinal center of the lymph node and can be subdivided into classical and lymphocyte predominant types

Question 7

A classical presentation of HL is ___ +/- B symptoms. The (presence/abscence) of B symptoms indicates a poor prognosis.

T/F: This cancer likes to travel to various parts of the body

Answer 7

A classical presentation of HL is lymphadenopathy +/- B symptoms. The presence of B symptoms indicates a poor prognosis.

Falsehood. HL tends to be limited to the lymph nodes (explains the lymphadenopathy) and tend to move in an orderly fashion from the LN >> spleen >> liver >> bone marrow

Question 8

HL is characterized by the presence of ___, which are actually a small percentage of the total number of cells in the lymph node

Answer 8

HL is characterized by the presence of Reed-Stenberg cells, which are actually a small percentage of the total number of cells in the lymph node

Question 9

The ___ subtype of Hodgkin’s lymphoma is characterized by B cell-derived cells that are __ and __ +ve (CD markers) and __ -ve despite being B cell derived. Some subtypes are also ___ +ve (a HHV that you will recall causes Hodgkin’s lymphoma)

Answer 9

The classical subtype of Hodgkin’s lymphoma is characterized by B cell-derived cells that are CD15 and CD30 +ve (CD markers) and CD20 -ve despite being B cell derived. Some subtypes are also EBV +ve (a HHV that you will recall causes Hodgkin’s lymphoma)

Question 10

The ___ subtype of Hodgkin lymphoma is characterized by CD20+ cells (CD15/30 and EBV -ve) and the prognosis is generally good

Answer 10

The non-classical/lymphocyte predominant subtype of Hodgkin lymphoma is characterized by CD20+ cells (CD15/30 and EBV -ve), “popcorn” RS cells and the prognosis is generally good

Question 11

Another characteristic symptom of Hodgkin’s lymphoma patients is __ if pts drink alcohol

Answer 11

Another characteristic symptom of Hodgkin’s lymphoma patients is extreme pain or itching if pts drink alcohol

Question 12

Contrast Hodgkin and Non-Hodgkin lymphoma in the table below

Answer 12

Question 13

___is a high grade form of Non-Hodgkin’s lymphoma that arises from cells in the germinal center or marginal zone

Answer 13

Diffuse B cell lymphoma is a high grade form of Non-Hodgkin’s lymphoma that arises from cells in the germinal center or marginal zone

Question 14

Which Non-Hodgkin Lymphoma is characterized by the pathology below?

Answer 14

**Diffuse large B cell lymphoma**

Pleomorphism; large nuclei

Mitotic activity

Form sheets in whatever organ they grow in

Question 15

Which gene abnormalities are seen in cases of DLBCL?

Answer 15

BCL2

BCL6

t(14;18)

Question 16

Immunoficiency associated DLBCL is a viral-ass’d cancer which can be caused by infection with which 2 viruses?

Answer 16

HIV

EBV-driven

**bone marrow transplantation is also a state of malignancy so there’s that**

Question 17

Primary effusin lymphoma is a type of DLBCL that arises from infection with ___ and is characterized by fluid involnement.

Answer 17

Primary effusion lymphoma is a type of DLBCL that arises from infection with HHV8/ HHV8+EBV co-infection and is characterized by fluid involnement (cancer usually involves pleural or perinotoneal fluid)

Question 18

Another type of aggressive NHL is ___

Answer 18

Another type of aggressive NHL is Burkitt lymphoma

Question 19

Burkitt lymphoma can be split into 3 subtypes, namely __ (all EBV+), ___ (more common in the Western hemisphere and generally EBV -) and ___ (may or may not be EBV+; its immunodeficient condition)

Answer 19

Burkitt lymphoma can be split into 3 subtypes, namely Endemic (all EBV+; more common in Africa), Sporadic (more common in the Western hemisphere and generally EBV -) and HIV+ (may or may not be EBV+; its immunodeficient condition)

Question 20

___ lymphoma is characterized by medium-sized cells w/ moderate cytoplasm, a “starry sky” appearance of macrophages laden with dead cells, high rate of mitosis + a rapidly growing mass

Answer 20

Burkitt lymphoma is characterized by medium-sized cells w/ moderate cytoplasm, a “starry sky” appearance of macrophages laden with dead cells, high rate of mitosis + a rapidly growing mass

Question 21

Which pathology is shown below?

Answer 21

Burkitt lymphoma

**The big cells with cytoplasm are macrophages that are eating up the dying cells as new ones proliferate rapidly**

Question 22

Burkitt lymphoma is CD__ and ___ +ve, and Bcl2 -ve. It also shows high expression of __ (marker of high rate of mitosis)

Answer 22

Burkitt lymphoma is CD10 and 20 +ve, and Bcl2 -ve. It also shows high expression of Ki-67 (marker of high rate of mitosis or in this ki-tosis)

Question 23

All forms of Burkitt Lymphoma has a translocation of the __ gene on chromosome 8

Answer 23

All forms of Burkitt Lymphoma has a translocation of the C-myc gene on chromosome 8.

t(8;14) – IgH locus: the gene translocates to and sits on the promoter of the Heavy chain locus

t(2;8) – Ig K (here the gene translocates to the Kappa promoter)

t(8;22) – Ig λ (gene translocates to the lambda promoter)

**know that C-MYC is a proto-oncogene**

Question 24

Low grade NHL can be subdivided into small lymphocytic lymphoma, ___ lymphoma, follicular lymphoma and ___

**hint: all of these lymphomas describe WHERE in the lymph node the cancer is happening**

Answer 24

Low grade NHL can be subdivided into small lymphocytic lymphoma, mantle cell lymphoma, follicular lymphoma and marginal zone

**hint: all of these lymphomas describe WHERE in the lymph node the cancer is happening**

Question 25

Small lymphocytic lymphoma can arise from the naïve B cell stage or the memory B cell stage in the marginal zone. Generally, the prognosis is worse with the ___ stage

Answer 25

Small lymphocytic lymphoma can arise from the naïve B cell stage or the memory B cell stage in the marginal zone. Generally, the prognosis is **worse with the naïve B cell stage**

Question 26

CLL/SLL tends to involve the entire RES system and causes high WBC count In the lymph node, disease is characterized by pale areas called ___ where the Ki-67 (mitotic rate) is higher than everywhere else In the blood, disease is characterized by lymphocytosis and ___ cells

Answer 26

CLL/SLL tends to involve the entire RES system; causes high WBC count In the lymph node, disease is characterized by pale areas called proliferation centers where the **Ki-67** (mitotic rate) is higher than everywhere else In the blood, disease is characterized by lymphocytosis and **“smudge” cells**

Question 27

\_\_\_ is a characteristic aberrant marker of CLL/SLL The presence of ___ is a marker of favorable prognosis

Answer 27

**CD5**\*\* is a characteristic aberrant marker of CLL/SLL \*\*also CD19 and CD20, CD23 +ve\*\* The presence of **somatic hypermutation** is a marker of favorable prognosis

Question 28

Mantle cell lymphoma is characterized by lymphadenopathy and spread to extra nodal sites (\_\_\_ in the tonsils, and ___ in the colon)

Answer 28

**Mantle cell lymphoma** is characterized by lymphadenopathy and spread to extra nodal sites (**Waldeyer ring** in the tonsils, and **lymphomatous polyposis** in the colon) \*\*Waldeyers ring: ringed arrangement of tonsils\*\*

Question 29

Mantle cell lymphoma is characteristically __ +ve and arises from a translocation involving ___ where the gene translocates to the promoter of the Ig heavy chain \*\*note that this disease is also CD5+ (and Cd19, 20 +ve)

Answer 29

Mantle cell lymphoma is characteristically **Cyclin D1 +ve** and arises from a translocation involving **Cyclin D 1** where the gene translocates to the promoter of the Ig heavy chain \*\*note that this disease is also CD5+ (and CD19, 20 +ve)\*\* \*\*t(11;14)

Question 30

\_\_\_ is a common indolent lymphoma that occurs in the lymph node germinal center

Answer 30

**Follicular lymphoma** is a common indolent lymphoma that occurs in the lymph node germinal center

Question 31

Which pathology is indicated below?

Answer 31

Follicular lymphoma

Question 32

What pathology is indicated below?

Answer 32

Follicular lymphoma

Question 33

Follicular lymphoma is characterized by the presence of which two cell types?

Answer 33

Centrocytes: small cells with cleaved nuclei Centroblasts: larger cells with prominent nucleoli

Question 34

In follicular lymphoma, there is a translocation that keeps ___ turned on and prevents the killing of these neoplastic cells

Answer 34

In follicular lymphoma, there is a translocation that keeps **Bcl2** turned on and prevents the killing of these neoplastic cells \*\*cells are Bcl2+, CD10, 19 and 20 +ve) \*\*t(14;18 translocation between bcl2 and the Ig heavy chain)

Question 35

Marginal zone lymphoma is characterized by abnormal proliferation of post-follicular B cells, so you may occassionally see what cell population with this cancer? What bacterial infection is this lymphoma ass'd with (hint: inflammation from this infection causes MALT lymphoma)?

Answer 35

Can occasionally see **plasma cells** ass’d with this lymphoma and is usually ass’d with **H/ pylori** infection (inflammation leads to **MALT lymphoma**) If you treat with antibiotics, the lymphoma goes away

Question 36

A characteristic finding of Marginal zone lymphoma is a __ lesion (neoplastic invasion of glandular tissue)

Answer 36

A characteristic finding of Marginal zone lymphoma is a **lymphoepithelial** lesion (neoplastic invasion of glandular tissue)

Question 37

Fill in the blanks

Answer 37