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Flashcards in Lymphoma Deck (61)
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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