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Flashcards in Lymphoma Deck (61):
1

What are lymphomas?

malignancies that develop from lymphocytes. The site of the maturation defines the type of lymphoma present

2

Clinically, what can lymphomas do to people?

-mass presence (LAD, ureteral obstruction, cord compression)
-replacement (pancytopenia)
-reduction (hypogammaglobinemia)
-tumor products (uric acid, calcium lysis)
-paraneoplastic (AHA, ITP, neuropathy)
-toxicity of therapy (infected, numb and short of breath)

-psychosocial/economic: broke and alone

3

What things does a physician need to know if order to treat a lymphoma patient?

-tumor histology
-tumor stage
-condition of patent
-available therapies

4

How is lymphoma histology divided clinically?

indolent
aggressive
highly aggressive
B or T cell

5

What are indolent (low grade) lymphomas?

slowly progressive, tend to be higher stage (more widespread), and tend to respond to simple therapies

tend to be incurable (except stage I-II)

6

T or F. Survival of indolent lymphoma is independent of early treatment

T. Treatment is more based on symptomatic presentation

7

What are some examples of indolent lymphomas?

-follicular (most common indolent)
-small lymphocytic/CLL
-MALT lymphomas
-Mycosis fungoides

not curable mostly

8

What do aggressive lymphomas look like?

often lower stage, rapidly progressive, requires complex therapies

tend to be curable

9

T or F. Survival of aggressive lymphoma is independent of early treatment

F. Early therapy required

10

What are some examples of aggressive lymphomas?

-diffuse large B cell
-mantle cell
-peripheral T cell lymphoma (NOS, anaplastic, angioimmunoblastic)

11

What are some examples of highly aggressive lymphomas?

-Burkitt/ Burkitt like
-T cell lymphoblastic lymphoma

12

What is a "stage 1" lymphoma?

single node or lymphoid structure

13

What is a "stage 2" lymphoma?

two+ lymph regions on one side of the diaphragm

14

What is a "stage 3" lymphoma?

lymph nodes on both sides of diaphragm

15

What is a "stage 4" lymphoma?

disease is extranodal beyond E

16

What is stage I/II E?

lymphoma originated in an extra nodal area and spread to adjacent lymph nodes

17

What are some tests used in staging lymphoma patients?

-H&P
-chest, abdomen, and pelvis CAT
-PET scan
-bone marrow aspirate and biopsy (CD20)
-CBC, LDH, check CSF

18

What does a PET scan allow?

irradiates nuclei so that you can see functional changes in lymph nodes in lymphoma whereas normal lymph nodes would not show up on the PET scan

19

What is the most common lymphoma?

diffuse large B cell (then follicular)

20

What is the International Prognosis Index?

Age>60 1
Stage III-IV 1
LDH elevated 1
P status >1 1
Extranodal sites > 1 1

APLES

Low risk: 0-1
Low intermediate risk: 2
High intermediate risk: 3
High risk: 4

5 yr. Survival
0-1 73%
2 51%
3 43%
4-5 26%

21

What is the Follicular lymphoma IPI?

Age > 60
Nodal sites > 4
LDH increased
Stage III-IV
Hemoglobin

0-1 = 90% 5 yr sv
2 = 77% 5 yr sv
3-5 = 52% 5 yr sv

22

What are some therapy options for indolent lymphomas?

remember, don't treat unless symptomatic

-local irradiation
-Alkylators and prednison
-Anthracyclines
-Fludarabine
-CD20 antibodies
-Combo chemo

23

What are some therapy options for aggressive lymphomas?

-combo chemo-CHOP; HyperCVAD
-antibody therapy plus CHOP (rituximab)
-radio-immunotherapy
-autologous BM transplant for relapse (survival advantage)

24

What is CHOP?

cyclophosphamide, hydroxydaunorubicin,
oncovin (vincristine),
prednisone

25

What is the antibody drug against CD20?

Rituximab- activates complement or phagocytosis

26

T or F. Adding rituximab to CHOp therapy for diffuse B cell lymphoma helps a lot

T. Almost always use R-CHOP therapy

27

What is the current therapy for stage I/II (IE/IIE) intermediate grade NHL?

CHOP-3 plus RT (stage 1-83% 5 yr survival; stage II-74%)

28

What is the current therapy for stage bulky II/IIE, III, and IV intermediate grade NHL?

CHOP-8

(stage II bulky -49% 5 yr survival; stage III/IV-46%)

29

What is the current therapy for first relapse for all stages of intermediate grade NHL?

autotransplant (53% 5 yr survival)
DHAP (32% 5 yr survival)

30

What drug targets CD52 (B and T cells)?

alemtuzumab

31

What drug targets CD30 (common on Hodgkin lymphoma)?

brentuximab vedotin

32

What drug targets BTK?

ibrutinib

33

What drug targets CD22?

inotuzumab ozogamicin

34

Describe the B cell receptor pathway

Antigen binding induces the aggregation of the BCR with its coreceptors CD79A and B, which become phosphorylated by the tyrosine kinases LYN and SYK. SYK activates phosphoinositide 3–kinase (PI3Kδ), which in turn converts phosphatidylinositol 4,5-bisphosphate (PIP2) to phosphatidylinositol 3,4,5-triphosphate PIP3. PIP3 serves as a docking site for the cytoplasmic kinases Bruton's tyrosine kinase (BTK) and AKT. BTK phosphorylates and thereby activates phospholipase C gamma 2 (PLCγ2), which in turn generates a set of second messengers to activate protein kinase C beta (PKCβ). PKCβ phosphorylates IκB kinase (IKK) to activate nuclear factor κB (NF-κB) transcription factors that regulate gene expression of several survival factors. The kinases inhibited by small molecules with promising clinical activity are indicated.

35

What drug inhibits SYK?

Fostamatinib

36

What drug blocks PI3Kδ?

Idelalazib (GS-1101)

37

What infections are associated with lymphoma?

HIV (primary CNS lymhpoma)
HHV8, (primary plueral effusion lymphoma)
HTLV-1, (T cell lymphomas-ATLL)
EBV, (Burkitt)
H. pylori (MALT type lymphomas of GI tract)

38

What organ does mycosis fungicides (MF) target primarily?

skin (when blood is involved- Sezary)

39

How is early stage MF treated?

Soralin (sp?) and UV light only

when disease becomes systemic, need radiation

40

What are some T cell lymphomas?

-Angioimmunoblastic T cell (AITL)
-Anaplastic large cell
-Adult T-cell leukemia/lymphoma (ATLL)

41

T or F. ALK+ is favorable in anaplastic large cell lymphoma patients

T. Mediastinal presentation in young adults

42

What is AITL associated with?

-hypergammaglobinemia
-AHA

43

What are the types of Hodgkin's disease?

1) lymphocyte predominant (in between indolent and aggressive B cell lymphoma)

2) Classical Hodgkin's
-lymphocyte rich
-nodular sclerosis
-mixed cellularity
-lymphocyte depleted (more aggressive)

44

What is the most common type of classical hodgkin's in children?

nodular sclerosis. As you get older, mixed cellularity and lymphocyte depleted become more common

45

How is Hodgkin's Disease different than NHL?

-Hodgkin is CD30+

progress differently (goes from one lymph node to the next)

same staging and tests

46

How is early stage Hodgkin treated?

Treat with short course chemotherapy and focal radiation to area of disease or radiation alone or chemo alone

47

How is late stage Hodgkin treated?

chemotherapy
-ABVD: adriomycin, bleomycin vinblastine, DTIC

-good prognosis

48

The problem with Hodgkin disease is that they live a long time and may develop complications (via chemo) like:

-Acute leukemia and myelodysplasia

-Solid tumors-breast, lung, stomach, bone, soft tissues

-Coronary artery disease

-Radiation and chemotherapy pneumonitis

-Infertility

-Hypothyroidism

49

What is chronic lymphocytic leukemia (CLL)?

-indolent B cell malignancy

50

patient population for CLL?

50+ y/o

51

Symptoms of CLL?

Often asymptomatic (may never need treatment)

Lymphocytosis
Lymphadenopathy
Splenomegaly
Anemia (from AHA)
Autoimmune cytopenias
Hypogammaglobulinemia

52

What is RAI staging for CLL?

0 = lymphocytosis
1 = enlarged lymph nodes
2= hepatosplenomegaly
3 = Hgb less than 10 (non-immune)
4 = Platelets less than 100,000 (non-immune)

53

What is Binet staging for CLL?

based on lymph node involvement:

A=0-2 areas
B=> 2 areas
C= Hgb less than 10 or platelets less than 100K

54

What are some negative prognostic indicators for CLL?

High Stage

Increased rate of lymphocyte doubling time (double in less than 6 months is bad)

Beta 2 microglobulin elevated (poor)

Deletion 17p, TP53 mutations, 11q deletion (very bad)

Unmutated VH genes, ZAP-70, CD 38

55

How is CLL treated?

Alkylator: Chlorambucil, cyclophosphamide, bendamustine

Purine analogues: Fludarabine

Chemotherapy combinations: FCR

Immunotherapy: rituximab (CD 20), alemtuzumab (CD 52), ibrutinib (if p53 mutation is present)

Radiation

Corticosteroids

56

What is Hairy cell lymphoma (HCL)?

indolent B cell lymph proliferative disorder

57

Are men or women more likely to be affected by HCL?

male (4:1)

58

Symptoms of HCL?

-splenomegaly
-pancytopenia
-decreased cell mediated immunity

59

Immunophenotype of HCL?

11c,19,20,22,25,103

60

What are the treatment indications for HCL?

Treatment Indications:
Cytopenias
ANC

61

Treatment for HCL?

Very high and enduring response rates to 2- chlorodeoxyadenosine and deoxycoformycin