Lymphoma

Question 1

EBV

Answer 1

Burkitt Lymphoma

Hodgkin Lymphoma

Question 2

HTLV-1

Answer 2

T cell leukemia and lymphoma

Question 3

HCV

Answer 3

Splenic Marginal Zone Lymphoma

Question 4

HIV

Answer 4

B-Cell Lymphomas

Question 5

Kaposi sarcoma herpesvirus

Answer 5

Kaposi sarcoma

Primary Effusion Lymphoma

Question 6

Sjögren Syndrome

Answer 6

Extranodal Marginal Zone Lymphomas

Question 7

Best diagnostic tool for Lymphoma

Answer 7

Excisional Biopsy > Core Needle Biopsy

Question 8

Basics of Ann Arbor Lymphoma Staging

Answer 8

Stage I: single lymph node region or single extralymphatic site (1E)
Stage II: more than 1 LN region on SAME side of diaphragm
Stage III: LNs on both sides of diaphragm; if Hodgkin (III1= upper abdomen, III2= lower abdomen)
Stage IV: disseminated extralymphatic Disease involving one or more organ

Question 9

Which lymphoma types do not necessarily need a bone marrow biopsy if PET is negative?

Answer 9

Hodgkin

Large Cell Lymphoma

Question 10

What do the lymphoma stages A and B mean?

Answer 10

B = symptoms of fevers, drenching night sweats or weight loss

Question 11

Are non-Hodgkin Lymphomas B- or T- Cell derived?

Answer 11

85% B cell derived

Question 12

List the Indolent B Cell Lymphomas

Answer 12

CLL
Hairy Cell Leukemia
MALT Lymphoma
Follicular

Question 13

What is the most common indolent B cell lymphoma?

Answer 13

Follicular Lymphoma

Question 14

Genetics of Follicular Lymphoma

Answer 14

t(14;18) leading to overexpression of BCL2 oncogene

Question 15

How is Follicular Lymphoma Treated?

Answer 15

Minority (presenting with local disease)— radiation therapy with curative intent

Majority (asymptomatic and advanced when presenting)— rituximab + chemo—> remission in ~90% but does not increase overall survival

Question 16

What is the dreaded complication of follicular lymphoma and how common is it?

Answer 16

30% transformation to Diffuse Large B cell Lymphoma

Question 17

How does gastric MALT therapy differ from MALT at other sites?

Answer 17

Treat with abx+ PPI

Usually radiation therapy alone leads to a good response

Question 18

What are the common B Cell markers?

Answer 18

C19,20,23

Question 19

How do you diagnose CLL?

Answer 19

Smear with increased lymphocytes (smudge cells), and flow cytometry with B cell markers and coexpressuon of CD5 (t-cell marker)

** do NOT need bone marrow biopsy

Question 20

What is the term for CLL with more lymphoma manifestation?

Answer 20

Small Lymphocytic Lymphoma

Question 21

What are two important considerations for patients with CLL/Small Lymphocytic Lymphoma?

Answer 21

(1) Relative immunosuppression — May need IVIG if getting lots of infections
(2) can get autoimmune cytopenias (ex ITP, autoimmune hemolytic anemia)

Question 22

What is the dreaded complication of CLL and how common is it?

Answer 22

Richter Transformation (transformation to large cell lymphoma)- in 5% of patients

Question 23

How does hairy cell leukemia present?

Answer 23

Cytopenias and Splenomegaly

Question 24

What genetic mutation is important in hairy cell leukemia?

Answer 24

BRAFV600E

Question 25

What is the standard treatment for hairy cell leukemia?

Answer 25

Purine Nucleoside Agents (pentostatin or cladribine) If BRAF mutation can use Vemurafenib

Question 26

List the aggressive B Cell Lymphomas

Answer 26

Diffuse Large B Cell Mantle Cell Burkitt

Question 27

What is the typical presentation for diffuse large B cell lymphoma?

Answer 27

Neck or abdomen enlarging adenopathy | 33% with systemic symptoms

Question 28

Biopsy results in DLBCL?

Answer 28

Large atypical lymphoid cells with prominent nucleoli and basophilic cytoplasm

Question 29

What is the typical treatment for diffuse large B cell lymphoma?

Answer 29

R-CHOP (usually advanced at presentation) +\- consolidators radiation

Question 30

What features are associated with a poor prognosis in diffuse large B cell lymphoma?

Answer 30

Elevated LDH, extensive tumor burden, poor performance status

Question 31

Who usually gets primary mediastinal large cell lymphoma? Which cells do these arise from?

Answer 31

Young females, arising from thymic B cells

Question 32

Who typically gets mantle cell lymphomas?

Answer 32

Men, median age 68

Question 33

What are the genetic mutations associated with mantle cell Lymphoma?

Answer 33

t(11;14) causing overexpression of cyclin D

Question 34

What is the genetic mutation associated with Burkitt lymphoma?

Answer 34

MYC gene activation

Question 35

What are the treatment considerations for Burkitt lymphoma?

Answer 35

(1) Tumor Lysis syndrome is anticipated | (2) treat for occult CNS involvement given risk for leptomeningeal involvement

Question 36

What is the typical treatment for Burkitt lymphoma?

Answer 36

Multi agent chemo with Rituximab

Question 37

What are the two types of cutaneous T-cell lymphoma?

Answer 37

Mycosis Fungoides | Sézary Syndrome

Question 38

What are the defining features of Sézary syndrome?

Answer 38

Diffuse Erythroderma | Malignant T cells circulating in blood

Question 39

What is the genetic mutation associated with anaplastic large cell lymphoma and why is it important?

Answer 39

t(2;5) = variant ALK gene translocation Can use Crizotinib

Question 40

How is lymphoblastic lymphoma treated?

Answer 40

Like ALL (very aggressive)

Question 41

How do you diagnose Hodgkin Lymphoma?

Answer 41

Bone marrow biopsy with Reed Sternberg cells (owl eyes)

Question 42

How do you stage Hodgkin Lymphoma?

Answer 42

PET and Physical Exam

Question 43

What is the most commonly used chemo regimen in the USA for Hodgkin Lymphoma?

Answer 43

``` ABVD +\- field radiation Doxorubicin Bleomycin Vinblastine Dacarbazine ```

Question 44

What may you do for relapsed Hodgkin lymphoma?

Answer 44

Allogenic Hematopoietic Stem Cell Transplant With Brentuximab Vedotin (anti-CD30 monoclonal Ab) consolidation

Question 45

What is important to do after 2 to 3 cycles of chemotherapy in Hodgkin lymphoma?

Answer 45

PET- if negative, may not need radiation

Question 46

How does nodular lymphocyte predominant Hodgkin lymphoma differ from classic Hodgkin lymphoma?

Answer 46

10% of cases, more likely to be localized, associated with higher rate of late relapse. Usually can treat with radiation therapy alone if early stage