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Flashcards in Small B Cell NHLs Deck (41):
1

what are small B cell NHLs derived from?

mature B cells

2

is bone marrow involvement common in small B cell NHLs?

yes. lots of stage 4 disease (as well as liver/spleen involvement)

3

immunophenotypic profile for follicular lymphoma

CD19/20+ (mature B cell)
CD5- (not T cell)
kappa/lambda restricted
CD10+, BCL6+, BCL2+ (germinal center origin, but cells don't apoptose)

4

immunophenotypic profile for CLL/SLL

CD19/20+ (mature B cell)
CD5+ (aberrant T cell marker)
kappa/lambda restricted
CD10-, BCL6-, BCL2+ (not from germinal center)

5

immunophenotypic profile for marginal zone lymphoma (MALT)

CD19/20+ (mature B cell)
CD5- (not T cell)
light chain restriction
CD10-, BCL6-, BCL2+ (not germinal center origin)
** no distinct immunophenotypic marker***

6

what is the most common NHL?

follicular lymphoma

7

what age group does follicular lymphoma affect?

middle age and older

8

morphology of follicular lymphoma

nodular neoplasm within lymph node. loss of mantle, polarity, tingible macrophages. complete loss of normal features. Contains centroblast & centrocytes.

9

growth of follicular lymphoma

indolent

10

clinical manifestation of follicular lymphoma

generalized lymphadenopathy, typically caught at stage 4 (disseminated) due to slow growth. spleen/liver involvement common with miliary lesions and painless splenomegaly. extra nodal involvement is uncommon.

11

prognosis of follicular lymphoma

incurable due to indolent growth (therapies target cell division). 7-9 year survival

12

current therapy for follicular lymphoma

observation, symptomatic relief, Rituxan, multiagent regiments (CHOP)

13

more severe follicular lymphoma

can transform into diffuse large b cell lymphoma. can occur after treatment and cuts survival to <1yr

14

derivation of follicular lymphoma

germinal center B cell

15

centrocytes

small cleaved, low grade germinal center b cells that can disseminate through the blood

16

centroblasts

large, transformed noncleaved germinal center B cells.

17

how to grade follicular lymphomas

1. Cytology: centroblast count
2. Extent of nodularity

(higher grades=higher centroblasts, diffuse nodules=more aggressive)

18

bone marrow pattern in stage 4 follicular lymphoma

paratrabecular aggregates

19

follicular lymphoma translocation

t(14;18). IgH-Bcl2=constitutive Bcl2 activity=no apoptosis

20

Ki67 in follicular lymphoma

low. cells aren't proliferating because they aren't dying. Just accumulate instead.

21

CLL/SLL derivation

unclear. either 1. mature B cells that just left marrow, haven't encountered antigen, no TdT/CD34, with light chains or 2. B cells that encountered antigen in follicle and are chilling in the margin

22

CLL/SLL stands for...

Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma (circulating cells and solid masses)

23

epidemiology of CLL/SLL

affects 60+ and males more than females. most common adult leukemia

24

clinical features of CLL/SLL

usually asymptomatic, generalized lymphadenopathy, always stage 4, spleen&liver involvement, peripheral blood involvement (unlike FL), immune dysregulation (AIHA or thrombocytopenia) caused by normal B cells

25

CLL/SLL prognosis

variable survival 4-6 years, easily transforms to prolymphocytic leukemia or undergoes Richter's Transformation (both <1yr survival and resistant to treatment)

26

Richter's Transformation

transformation of CLL/SLL to diffuse large B cell lymphoma

27

CLL/SLL morphology

diffuse sheet of small resting lymphocytes in lymph node with proliferation centers of larger transformed cells with more cytoplasm (pro lymphocytes or paraimmunoblasts). No follicle structure, effacement of architecture

28

peripheral blood smear of CLL/SLL

lymphocytosis, smudge cells (disrupted white cells), spherocytes (autoimmune attack), nucleated RBCs, increased reticulocytes (polychromasia)

29

CLL/SLL bone marrow involvement

nodular aggregates (or diffuse spread) in marrow away from trabecular in area of interstitium

30

naive form of CLL/SLL

contains rearranged but unmutated Ig, pre-germinal center, Zap70+, aggressive form of the disease

31

post germinal center form of CLL/SLL

contains rearranged and somatically hyper mutated Ig genes, has traversed GC. Zap70-, Indolent

32

Marginal Zone Lymphoma origin

comes from ill defined area outside germinal center where B cells go after encountering antigen and either become memory or plasma B cells.

33

three separate marginal zone lymphoma diseases

Nodal, Muscosal/Extranodal (MALT), Splenic

34

MALT stands for...

extranodal marginal zone lymphoma of mucosa associated lymphoid tissue

35

Cause of MALT

chronic inflammatory lesions (helicobacter, campylobacter, ect)

36

MALT prognosis

indolent, but still can transform to diffuse large B cell lymphoma

37

sites of MALT

Stomach, Small bowel, skin, orbit, Lung, Parotid, thryoid

38

MALT morphology

abnormal germinal centers with expanded margins, and effacement of underlying architecture. small mature lymphocytes beyond margin, monocytoid b cells in margin, ample plasma cells

39

consequence of increased plasma cells in MALT

can secrete a low level of Ig into serum and urine (paraprotein)

40

main mechanism of MALT lymphomas

inhibit apoptosis/promote cell growth via NF-kB activation

41

treatment of MALT

can treat underlying infections up to a certain point and neoplasm will regress. eventually cellular change will be independent of inflammatory stimulus and chemotherapy is required