Chart 1

Low-grade NHL (all

lymphomas)

Origin of
neoplastic
cells

Small lymphocytic

lymphoplasmacytoid
(LPL) AND
Waldenstroms
macroglobulinemia
(latter is LPL
involving monoclonal
IgM paraprotein)

-postgerminal
center B
cell (has
charcteristic
s of IgMbearing
memory B
cell)

Marginal zone

-marginal
zone of Bcell
germinal
follicles
(spleen,
MALT,
lymph
nodes)

Follicular (most
common NHL)

Distinguishing clinical features

-same morphology, ummunophenotype,
cytogenetics and chronic lymphocytic lymphoma
(CLL)
-less than 5x109/L peripheral blood B cells
-no cytopenias due to bone marrow involvement
- insidious onset
-neuro sx, dyspnea, HF (presenting sx)
- fatigue and weight loss
-hyperviscosity syndrome
-anemia (caused by increase plasma volume)
-bleeding tendency (caused by macroglobulin
interfering w/coagulation factors and platelet
function)
-moderate lymphadenopathy, splenomegaly,
hepatomegaly
-pt might be asx
-splenomegaly, associated w/circulating villous
lymphocytes

-benign course for many years (present as polyps in

duodenum, in skin or in kids but 3% suddenly
transform into aggressive diffuse tumors every
year)
-painless lymphadenopathy
-widespread
-most pts have stage III or IV disease (graded
according to relative proportion of centrocytes and
centroblasts among other things)
-usually bone marrow involvement
Other info: Associated w/translocation (t(14;18))
that leads to constitutive expression of BCL-2 gene
reduced apoptosis of cells increased cell
survival

Mantle cell

-pregerminal

-lymphadenopathy w/bone marrow involvement (like

follicular)

Age
of
onset
Elderl
y

Treatments

Men
over
50 y/o

-if pt asx, no tx
-otherwise, use rituximab IN COMBO w/
cyclophosphamid, fludarabine or other purine
analog, bendamustine or bortezomib (rituximab is
humanized monoclonal antibody to CD20, omit
combo if hyperviscosity)
=erythropoietin or regular transfusions for chronic
anemia
-late stage: combination chemotherapy (follicular
or large cell diffuse B lymphoma) or stem cell
transplant
-splenectomy
-chemotherapy (use regimen normally used for
other low-grade lymphomas)
-radiotherapy
-rituximab

-60
y/o

-stage I (10% pts): localized; use radiotherapy

alone to cure
-stages II-IV: disseminated; if asx no tx, watch and
wait; when complications occur, then use tx.
Chemo NOT curative.
-Give monthly courses of R-CVP (rituximab in
combination w/cyclophosphamide, vincristine, and
prednisolone) OR give rituximab w/bendamustine
or chlorambucil (either way, 90% remission for
several years.
-rituximab infusions as maintenance therapy
every 2-6 months (cuz Ab has long half life)
-after inevitable relapse: tx w/similar chemo
followed by rituximab maintenance but when
harder to control use more intense chemo or
radiolabelled antibody therapy (anti CD-20); maybe
stem cell transplant too
-mean survival 10 years from dx (which is delayed)
-R-CHOP
-intensive combinations like R-Hyper-CVAD

None (watch and wait)

center cells
localized in
primary
follicles

-indolent course similar to CLL

(characteristic t(11;14) translocation, cells w/angular
nucleisee above for details)
Other info: cells have increased
expression of cyclin D1 which is needed to
make a dx
Cells show angular nuclei (pic to right)
-in chain disease: start as malabsorption
syndrome, progress to systemic lymphoma

Heavy chain diseases

-cytosine arabinoside, rituximab, autologous stem

cell transplant
-purine analogues w/ or w/o cyclophosphamide and
rituximab
-overall survival is 4-6 years

-no age of onset but chain disease mostly in

Mediterranean area

Chart 2

High-grade NHL (all

lymphomas)

Origin (if
applicable
)

Prognosis

Treatment

Diffuse large B cell

long term survival

65%

-rituximab in combo w/CHOP chemo (cyclophosphamide,

hydroxodaunorubicin, vincristine and prednisolone) in 2-3week cycles for 6-8
months (note this is mainstay tx but works best for localized diseases)
-G-CSF to support neutrophil count
- in high risk-disease: methotrexate as prophylactic for CNS disease
-for relapse: high dose chemo w/ESHAP (etoposide, cytosine, arabinoside,
methylprednisolone and cisplatin) OR R-ICE (rituximab, ifosfamide,
carboplantin and etoposide) (if respond, do autologous stem cell transplant)
-outcome depends on age, performance status, stage, number of extranodal
sites and serum LDH
-if bulky disease (mass>5 cm in diameter) and prior hx of low-grade disease
or AIDSpoorer prognosis

Burkitt

Excellent

-initially very responsive to chemo, but long-term cute is rare

-chemo should include high-doses of methotrexate, cytosine arabinoside and
cyclophosphamide (ie. CODOX-M/IVA which includes doxorubicin, ifosphamide
and etoposide)
-can give chemo intrathecal too
-DA-Epock is infusion regimen that you can use

Other info:
-Important to note
Endemic/African
form seen in areas
w/chronic malaria
exposure
-Associated w/ EBV
infection (African
form and sporadic
form)
-pt usually a kid
with a big jaw from
lymphadenopathy
Lymphoblastic

B or T cell

-same tx as ALL