15 - Lymphoproliferative Disorders 1 Flashcards
most common type of leukemia in western world
CLL (1/3 of leukemias)
CLL epi - age, gender
older, male
most common type of genetic mutation assoc w/ CLL
deletion
CLL cells tend to have high levels of ___
Bcl-2 - anti apoptotic
dx of CLL
persistent monoclonal lymphocytosis
PBS
(dont need bone marrow)
clinical presentation of CLL
asymptomatic lymphocytosis \+/- LAN, splenomegaly recurrent infxns anemia, thrombocytopenia w/ advanced dz B sx, fatigue, anorexia are rare
B symptoms
fever, night/cold sweats, unintentional wt loss >10% in 6 mo or less
possible autoimmune complications of CLL
autoimmune hemolytic anemia
thrombocytopenia (ITP)
pure red cell aplasia
Rai staging for CLL
0 - leukocytosis only I - LAN II - splenomegaly III - anemia IV - thrombocytopenia
Binet staging for CLL
A: = 3 nodal areas
C: anemia or thrombocytopenia
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
in what type of leukemia do you not need to treat the high WBC count?
CLL
tx of CLL
chemo - chlorambucil, bendamustine, cyclophosphamide, fludarabine
ab - rituximab, alemtuzumab (anti CD52)
use combinations
richter transformation
transition from indolent leukemia to aggressive lymphoma. occurs in about 1-10% of CLL pts
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)
t(14,18) is assoc w/
most follicular lymphoma and some higher grade NHL
t(11,14) is assoc w/
all mantle cell lymphoma
t(8,14), t(2,8), t(8,22) [c-Myc] assoc w/
Burkitt lymphoma
clinical features of NHL
LAN (may fluctuate) splenomegaly, hepatomegaly B sx cytopenias organ dysfunction rarely autoimmune problems
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
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
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
2 most common NHL
diffuse large b cell
follicular
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
small lymphocytic lymphoma
B cells
CLL but in lymph nodes
MALT lymphoma = Marginal zone lymphoma
mostly in stomach (H pylori), but any mucosa can be affected
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
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
diffuse large B cell lymphoma
aggressive NHL, most common
LDH usually elevated
more younger patients that the indolent forms, still mostly old though
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
tx for advanced aggressive NHL
can be curable systemic chemo (R-CHOP first line) may add intrathecal chemo or radiotherapy
R-CHOP
rituximab cyclophosphamide hydroxydaunorubicin oncovin (vincristine) prednisone
useful for NHL
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
