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Hem/Onc > 15 - Lymphoproliferative Disorders 1 > Flashcards

15 - Lymphoproliferative Disorders 1 Flashcards

1
Q

most common type of leukemia in western world

A

CLL (1/3 of leukemias)

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2
Q

CLL epi - age, gender

A

older, male

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3
Q

most common type of genetic mutation assoc w/ CLL

A

deletion

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4
Q

CLL cells tend to have high levels of ___

A

Bcl-2 - anti apoptotic

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5
Q

dx of CLL

A

persistent monoclonal lymphocytosis
PBS
(dont need bone marrow)

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6
Q

clinical presentation of CLL

A 
asymptomatic lymphocytosis
\+/- LAN, splenomegaly
recurrent infxns
anemia, thrombocytopenia w/ advanced dz
B sx, fatigue, anorexia are rare
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7
Q

B symptoms

A

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

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8
Q

possible autoimmune complications of CLL

A

autoimmune hemolytic anemia
thrombocytopenia (ITP)
pure red cell aplasia

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9
Q

Rai staging for CLL

A 
0 - leukocytosis only
I - LAN
II - splenomegaly
III - anemia
IV - thrombocytopenia
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10
Q

Binet staging for CLL

A

A: = 3 nodal areas
C: anemia or thrombocytopenia

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11
Q

when do you treat CLL?

A

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

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12
Q

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

A

CLL

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13
Q

tx of CLL

A

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

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14
Q

richter transformation

A

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

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15
Q

4 common etiologies of NHL

A

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

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16
Q

t(14,18) is assoc w/

A

most follicular lymphoma and some higher grade NHL

17
Q

t(11,14) is assoc w/

A

all mantle cell lymphoma

18
Q

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

A

Burkitt lymphoma

19
Q

clinical features of NHL

A 
LAN (may fluctuate)
splenomegaly, hepatomegaly
B sx
cytopenias
organ dysfunction
rarely autoimmune problems
20
Q

staging workup for NHL

A 
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
Q

staging of NHL

A

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
Q

indolent vs aggressive lymphoma - general course/prognosis

A

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
Q

2 most common NHL

A

diffuse large b cell

follicular

24
Q

follicular lymphoma

A 
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
Q

small lymphocytic lymphoma

A

B cells

CLL but in lymph nodes

26
Q

MALT lymphoma = Marginal zone lymphoma

A

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

27
Q

management of indolent NHL

A

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
Q

tx options for advanced indolent NHL

A

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

can also do immunotherapy alone, radioimmunotherapy, radiation alone

29
Q

diffuse large B cell lymphoma

A

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

30
Q

tx for early stage aggressive NHL

A

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
Q

tx for advanced aggressive NHL

A 
can be curable
systemic chemo (R-CHOP first line)
may add intrathecal chemo or radiotherapy
32
Q

R-CHOP

A 
rituximab
cyclophosphamide
hydroxydaunorubicin
oncovin (vincristine)
prednisone

useful for NHL

33
Q

burkitt lymphoma

A

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

Hem/Onc (29 decks)

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