15 - Lymphoproliferative Disorders 1 Flashcards Preview

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Flashcards in 15 - Lymphoproliferative Disorders 1 Deck (33):
1

most common type of leukemia in western world

CLL (1/3 of leukemias)

2

CLL epi - age, gender

older, male

3

most common type of genetic mutation assoc w/ CLL

deletion

4

CLL cells tend to have high levels of ___

Bcl-2 - anti apoptotic

5

dx of CLL

persistent monoclonal lymphocytosis
PBS
(dont need bone marrow)

6

clinical presentation of CLL

asymptomatic lymphocytosis
+/- LAN, splenomegaly
recurrent infxns
anemia, thrombocytopenia w/ advanced dz
B sx, fatigue, anorexia are rare

7

B symptoms

fever, night/cold sweats, unintentional wt loss >10% in 6 mo or less

8

possible autoimmune complications of CLL

autoimmune hemolytic anemia
thrombocytopenia (ITP)
pure red cell aplasia

9

Rai staging for CLL

0 - leukocytosis only
I - LAN
II - splenomegaly
III - anemia
IV - thrombocytopenia

10

Binet staging for CLL

A: = 3 nodal areas
C: anemia or thrombocytopenia

11

when do you treat CLL?

observation if asymptomatic
tx for progressive dz, B sx, cytopenias
if cytopenia is due to autoimmune, may be able to treat that on its own (steroids) and leave CLL alone

12

in what type of leukemia do you not need to treat the high WBC count?

CLL

13

tx of CLL

chemo - chlorambucil, bendamustine, cyclophosphamide, fludarabine
ab - rituximab, alemtuzumab (anti CD52)
use combinations

14

richter transformation

transition from indolent leukemia to aggressive lymphoma. occurs in about 1-10% of CLL pts

15

4 common etiologies of NHL

immunosuppression
DNA repair defects (ataxia telangiectasia, xeroderma pigmentosum)
viruses (EBV, HTLV-1, HCV, HHV8)
chronic inflammation (H pylori, sjogren syndrome, hasimoto thyroiditis)

16

t(14,18) is assoc w/

most follicular lymphoma and some higher grade NHL

17

t(11,14) is assoc w/

all mantle cell lymphoma

18

t(8,14), t(2,8), t(8,22) [c-Myc] assoc w/

Burkitt lymphoma

19

clinical features of NHL

LAN (may fluctuate)
splenomegaly, hepatomegaly
B sx
cytopenias
organ dysfunction
rarely autoimmune problems

20

staging workup for NHL

CBC, chemistries, urinalysis
CT of chest/abd/pelvis
PET scans being used more often
BM biopsy and aspirate
LP for T cell lymphoblastic lymphoma, aggressive lymphoma with pos marrow, AIDS lymphoma

21

staging of NHL

I - 1 LN region/structure
II - >1 LN, same side of diaphragm
III - both sides of diaphragm
IV - extranodal sites beyond E designation (ex BM, liver)

A - no B sx
B - B sx
E - single extranodal site
S - spleen
X - bulky

22

indolent vs aggressive lymphoma - general course/prognosis

indolent - life expectancy in yrs untreated
most present in stage 3-4
incurable in adv stages
more common in older people

aggressive - life expectancy in wks untreated
potentially curable even in adv stages
B sx more frequent

23

2 most common NHL

diffuse large b cell
follicular

24

follicular lymphoma

B cells
paradigm of indolent lymphoma
median survival 8-10 yrs
~30% transform to more aggressive
path - looks like tones of germinal centers/follicles all on top of each other

25

small lymphocytic lymphoma

B cells
CLL but in lymph nodes

26

MALT lymphoma = Marginal zone lymphoma

mostly in stomach (H pylori), but any mucosa can be affected

27

management of indolent NHL

stage 1 or 2 - can be cured w/ local radiotherapy
stage 3 or 4 - incurable and tx doesnt prolong survival, just observe and manage any sx that arise

28

tx options for advanced indolent NHL

1st line - chemoimmunotherapy
rituximab + cyclophos, vincristine, oncovin, prednisone, bendamustine (R-CVP, R-B, R-CHOP)

can also do immunotherapy alone, radioimmunotherapy, radiation alone

29

diffuse large B cell lymphoma

aggressive NHL, most common
LDH usually elevated
more younger patients that the indolent forms, still mostly old though

30

tx for early stage aggressive NHL

about half caught in stage 1 or 2
potentially curable
must use systemic chemo - R-CHOP 6 cycles, or R-CHOP 3 cycles followed by radiation

31

tx for advanced aggressive NHL

can be curable
systemic chemo (R-CHOP first line)
may add intrathecal chemo or radiotherapy

32

R-CHOP

rituximab
cyclophosphamide
hydroxydaunorubicin
oncovin (vincristine)
prednisone

useful for NHL

33

burkitt lymphoma

highly aggressive B cell NHL
African variety - jaw tumor, strongly linked to EBV
about half of cases in US linked to EBV
most rapidly growing human tumor
may present as abd mass
tx w/ multidrug regimen like pediatric leukemia/lymphoma regimens