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HemOnc Week 3 > ICL 11.1: Lymphoma I > Flashcards

ICL 11.1: Lymphoma I Flashcards

1
Q

what is lymphoma?

A

malignancy of lymphocytes that typically begins in the lymph nodes

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2
Q

where do lymphomas originate?

A

~2/3 Actually arise in lymph nodes

~1/3 of cases arise in extra nodal sites
like the GI tract, CNS, skin, eyes

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3
Q

what are the two major categories of lymphomas?

A
  1. Non-Hodgkin lymphoma

B cell (most common), T cell, and NK cell lymphomas

  1. Hodgkin Lymphoma

5 subtypes, WHO classification

classic and non-classic

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4
Q

what’s the difference between lymphoma and leukemia?

A

both are malignancies of the hematopoietic and lymphoid tissues…

but lymphomas are a malignancy of lymphoid cells that typically begins in the lymph nodes

and leukemias are a malignancy in either lymphoid or myeloid cells that begin in bone marrow

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5
Q

are lymphomas mono or polyclonal?

A

all lymphomas are derived from a single transformed cell and thus are monoclonal

B and T cell tumors are composed of cells derived from specific stages of their normal differentiation pathways

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6
Q

which lymphomas are derived from germinal center B cells?

A

follicular lymphoma

burkitt lymphoma

diffuse large B cell lymphoma

hodgkin lymphoma

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7
Q

which lymphomas are derived from precursor B cells?

A

B lymphoblastic leukemia/lymphoma

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8
Q

which lymphomas are derived from pre-GC B cells?

A

mantle cell lymphoma

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9
Q

which lymphomas are derived from post-GC B cells?

A

marginal zone lymphoma (MALT)

lymphoplasmacytic lymphoma

CLL/SLL

DLBCL

plasmacytoma

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10
Q

which lymphomas are derived from precursor T cells?

A

T lymphoblastic lymphoma/leukemia

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11
Q

where are B cells normally located in the lymph node?

A

cortex

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12
Q

where are T cells normally located in the lymph node?

A

paracortex

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13
Q

where are plasma cells normally located in the lymph node?

A

medulla

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14
Q

what happens to B cells once they’ve matured in the BM?

A

Naïve B-cell lymphocyte, move from bone marrow to lymph node cortex

they congregate in germinal follicle until they’re expose to antigens

naïve B cells in the germinal center proliferate, undergo somatic hypermutation, and differentiate into plasma cells

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15
Q

what are the different parts of the lymph node?

A

slide 11

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16
Q

explain the progression of the B cell through the lymph node dark and light zone

A
  1. naive B cell enters dark zone
  2. undergoes clonal expansion
  3. undergoes somatic hypermutation
  4. B cell enters light zone

the cells that underwent disadvantageous mutations get turned into apoptotic cells in the light zone and are destroyed

the B cells that had advantageous mutations get turned into plasma cells or memory B cells

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17
Q

what’s the clinical presentation of lymphoma?

A

Fever, night sweats, weight loss (any malignant disease)

Painless lymphadenopathy

Splenomegaly, hepatomegaly or mass

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18
Q

what’s the Ann Arbor staging system for lymphomas?

A

Stage 1: localized disease, single lymph node region or single organ

Stage 2: two or more lymph node regions on the same side of the diaphragm

Stage 3: two or more lymph node regions above and below the diaphragm

Stage IV: widespread disease, multiple organs, with or without lymph node involvement

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19
Q

what’s the Lugano classification system of lymphomas?

A

bulk ln Hodgkin lymphoma is defined as a mass greater than one-third of the thoracic diameter on CT of the chest or a mass >10 cm

for NHL, the recommended definitions of bulk vary by lymphoma histology

stages 1-4

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20
Q

how can you diagnose a lymphoma?

A

Fine-needle aspirate is inadequate for initial diagnosis. Excisional biopsy is recommended. Core-needle biopsy may suffice when excision not feasible

flow cytometry would help you with immunophenotyping

FISH would help you see translocations

PET and CT scans

***morphology is required to establish diagnosis

21
Q

what is the philadelphia translocation?

A

t(9;22) seen in CML

contains afusion genecalledBCR-ABL1

this causes a tyrosine kinasesignalingprotein that is “always on”, causing the cell todivideuncontrollably by interrupting the stability of the genome and impairing various signaling pathways governing the cell cycle

presence of this translocation is a highlysensitivetest for CML, since all cases of CML are positive forBCR-ABL1

However, the presence of the Philadelphia chromosome is not sufficientlyspecificto diagnose CML, since it is also found inacute lymphoblastic leukemia and occasionally inacute myelogenous leukemia as well as mixed-phenotype acute leukemia (MPAL).

22
Q

what is the Deauville 5-point scoring system?

A

an internationally accepted and utilized five-point scoring system for the Fluorodeoxyglucose (FDG) avidity of a Hodgkin’s lymphoma or Non-Hodgkin’s lymphoma tumor mass as seen on FDG

1: no 18-FGD uptake
2: uptake < mediastinal blood pool
3: uptake > mediastinum and < liver
4: uptake moderately > liver at any site
5: uptake markedly > liver at any site and/or new sites of disease

X: new areas of uptake unlikely to be related to lymphoma

23
Q

what are the main characteristics of NHL?

A

B-cell or T-cell

Mostly malignant cells

Widespread disease at Dx

Waldeyers ring common site

Noncontiguous spread

Low grade types incurable

24
Q

what are the main characteristics of HL?

A

Unique Reed-Sternberg cell (CD15 and CD30 +)

Many reactive cells, few malignant cells

Localized disease

Waldeyers ring rarely involved

Predictable contiguous spread

Generally curable

25
Q

what cells are involved in HL?

A

NOT B- or T-cell by immunostains or flow cytometry

yet, thbimodal age distribution

ought to be germinal or post-germinal B cell origin

26
Q

what population is HL most common in?

A

most patients are diagnosed peak between 15-30 age followed by another peak after 55 years of age

27
Q

is there a good or bad prognosis for HL?

A

good prognosis

often curable with combined modality therapy (chemotherapy and radiation)

long term survival is dependent on stage

28
Q

what diseases are HL patients at risk for?

A

At risk for AML, MDS, secondary solitary cancers including lung, thyroid and breast cancer, as well as cardiotoxicity

29
Q

what is the immunophenotype for classical HL?

A

CD20-

CD15+, CD30+, PAX5+

30
Q

what are the types of classical HL?

A
  1. nodular scelorisis
  2. mixed cellularity
  3. lymphocyte rich
  4. lymphocyte depleted
31
Q

what is the immunophenotype for non-classical HL?

A

CD20+

CD15-, CD30-

32
Q

what’s the immunophenotype for popcorn cells?

A

CD20+

CD15-

CD30-

they’re found in non-classical HL

slide 29

33
Q

what cell type is involved in NHL?

A

85-90% are B cells

34
Q

what are the different types of patterns seen in NHL?

A
  1. follicular pattern = always B cell
  2. diffuse pattern = B or T cell
  3. starry sky pattern = high grade lymphoma, especially Burkitt’s
35
Q

what population is NHL most common in?

A

usually older than 60

EXCEPT for lymphoblastic lymphoma = teens/20s and Burkitt lymphoma which is in kids

36
Q

what is the A and B distinction in the Ann Arbor staging system of lymphomas?

A

A = no systemic symptoms present

B = unexplained fevers, night sweats, weight loss

***Lugano modification of the Ann Arbor staging doesn’t use the A and B distinction

37
Q

is diffuse large b-cell lymphoma HL or NHL?

A

NHL

it’s the most common form of non-Hodgkins lymphoma

38
Q

what is DLBCL?

A

diffuse large B-cell lymphoma

agressive - painless, rapid growing mass

arises from B lymphocytes

extra nodal involvement is not unusual = liver, spleen GI tract, Waldeyers ring

immunocompromised, most common HIV related lymphoma

39
Q

what population is DLBCL most common in?

A

60-65 years

40
Q

what pattern is DLBCL?

A

diffuse pattern

normal architecture of lymph node replace by random pattern of malignant B cells

41
Q

what’s the immunophenotype for DLBCL?

A

CD19 +

CD20+

CD10 +/-

42
Q

which disease is DLBCL associated with?

A

HIV

DLBCL is immunodeficiency-associated

it’s seen in HIV or organ transplant patients due to severe T-cell decrease

malignant B-cells are usually infected with EBV

43
Q

what is follicular lymphoma?

A

indolent course but incurable

enlarging adenopathy in neck, axilla or groin

spreads to other lymph nodes, then extranodal sites and eventually BM

can transform to more aggressive, DLBCL

44
Q

what mutation is associated with follicular lymphoma?

A

t(14;18)

BCL-2 over activated with translocation, which blocks apoptosis

slide 44

45
Q

what is the immunophenotype of follicular lymphoma?

A

CD10

CD19

CD20

46
Q

what population is follicular lymphoma most common in?

A

60 years and up

47
Q

what are the different grades of follicular lymphoma?

A

grade 1 = small cells

grade 2 = mixed small and large cells

grade 3 = mostly large cells

small cleaved cells = centrocytes

large non-cleaved cells = centroblasts

48
Q

what are signs of a poor prognosis for follicular lymphoma?

A

B symptoms

metastasis

higher grade (large cells)

HemOnc Week 3 (22 decks)

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