Lymphomas- Clinical Presentation And Diagnosis

Front 1

what is the most common form of Non-hodgkins lymphoma

Back 1

Diffuse Large B cell lymphoma-
DLBCL
(followed by follicular lymphoma FL)

Front 2

what are the indolent NHL's? (slow growing 95% 5 yr surv)

Back 2

MALTL
CLL/SLL

Front 3

what are the aggressive NHLs?
(rapid growing, 50% 5 yr surv)

Back 3

DLBCL*
MCL- mantle cell
PTCL- peripheral T cell lymphomas

Front 4

what are the very aggressive NHLs?
(extremely rapidly growing, you will survive 6 mo to 2 yrs)

Back 4

LL
BLL
(lymph node will double in size in 24 hours.)

Front 5

Why does follicular cell lymphoma have multiple colors in the pie chart?

Back 5

Grade III is aggressive and gets treated like the DLBCL.
Grades I and II are low grade lymphomas

Front 6

NHL risk factors

Back 6

-Incidence higher in men
-Higher in whites except
Blacks 25-54
-Increased risk in families with HL/NHL
-Immunosuppression, particularly organ transplant recips

Front 7

initial evaluation of NHL

Back 7

-Physical examination, attention to peripheral nodes, liver, spleen, Waldeyer’s ring
-Complete blood count, blood smear
? Flow cytometry- use antibodies to detect various surface antigens
-Liver function tests; LDH*; uric acid
-CT scans of (chest), abdomen, pelvis
-Bone marrow biopsy/aspirate
-PET scan in aggressive lymphomas
Other studies as indicated

Front 8

why do we do a SPEP laboratory test for NHL?

Back 8

The serum protein electrophoresis (SPEP) test measures specific proteins in the blood to help identify some diseases. Some subtypes of lymphomas present with elevation of various immunoglobulins (IgM). This could make blood have a higher viscosity, risk of strokes!!

Front 9

what diseases are ass w NHL?

Back 9

Sjögren’s disease
RA
SLE
H.pylori
--MALToma. you can treat this lymphoma with antibiotics!!

Front 10

Clinical presentation
of NHL

Back 10

-Lymphadenopathy (67%)
-Bone marrow involvement (32%)
-Extranodal involvement
Skin, GI, Bone
-Fever, night sweats, weight loss (B symptoms)
-Asymptomatic
-Obstruction due to lymphadenopathy
Ureters, bowel….
(lymph nodes can block these things.)

Front 11

Diagnosis
of NHL

Back 11

GOOD PATH SPECIMEN-excisional biopsy!!
Bone marrow biopsy
Flow cytometry
cytogenetics

Front 12

NHL staging is the same as HL. Techniques?

Back 12

CT scans
PET scans

Front 13

Characteristics of Indolent NHLs

Back 13

-Generally disseminated at diagnosis bc pts dont know they have it.
-Lymph nodes, spleen, bone marrow, peripheral blood
-Small lymphocytes, usually of B-cell lineage
-Relatively long median survival
-Sensitive to chemotherapy and radiotherapy, it goes away with the treatment. the problem is it comes back usually around 18-24 months.

Front 14

NHL- follicular lymphoma

Back 14

Indolent
Peaks in 5th & 6th decades
Men = women
White>black=Asians
T(14:18) with BCL2 gene rearrangement

Front 15

follicular lymphoma gene rearrangement

Back 15

T(14:18) with BCL2 gene rearrangement

Front 16

FLIPI

Back 16

survival curve for pts with follicular lymphoma (international prognostic index).
0/1- good survival
2/3 of the prognostic factors in the index- survival drops
4/5-survival really drops

Front 17

what goes into the FLIPI?

Back 17

-number of nodal sites (>4)(this can mean two cervical lymph nodes, one on the right and one on the left. just because they are both cervial does not mean they are one site.)
-LDH level (elevated)
-age (>60 yrs)
-stage (III-IV)
-hemoglobin level < 120 g/dl

Front 18

treatment for NHL follicular lymphoma

Back 18

Watch and Wait (worry)
Chemotherapy (combination CVP), especially if there is a doubling time of the lymph nodes of less than a year, or when pts are symptomatic (b symptoms)
Rituximab
Bortezomib
Bendamustin
Radioimmunotherpay (Zevalin, Bexxar)

Front 19

what is rituximab for follicular lymphoma?

Back 19

a monoclonal antibody anti cd20. not a traditional chemo, no hairloss, n/v, or bp drop. destroys b cell lymphomas.

Front 20

DCBCL

Back 20

Most common variant of aggressive NHL
40% of all B cell lymphomas
C-MYC in 5-15%

Front 21

treatment for DCBCL (NHL)

Back 21

R-CHOP (the R stands for Rituximab)
Bone Marrow Transplant for Recurrent Disease

Front 22

IPI for aggressive lymphomas

Back 22

(APLES)
-age >60 yrs
-performance status >1
-LDH high
-extranodal sites >1
-stage III or IV
( if you have 0 or 1, low. 2 low-intermed, 3 high intermed, 4 or 5, high.)

Front 23

Burkitt’s lymphoma (very Aggressive)
(NHL)

Back 23

-Derived from post germinal center B cells
-Rapid cellular proliferation
-Spontaneous cell death
-Starry sky appearance
Translocation of c-MYC
T(8:14)
Proliferation indices of near 100%

Front 24

Burkitt's Translocation

Back 24

Translocation of c-MYC
T(8:14)

Front 25

endemic burkitts?

Back 25

in Africa, ass. w/ EBV
(also, immunosuppressed are at risk)

Front 26

Burkitts treatment

Back 26

-High dose chemo
-Intrathecal chemotherapy (within the spinal canal)
-Bone marrow transplant
-Can be cured w high dose chemotherapy

Front 27

Radioimmunotherapy in Non-Hodgkin’s Lymphoma

Back 27

radiolabel an antibody. goes where the lymphoma cells are. doesnt have to hit every single cell in a lymph node aggregate!! one or 2 antibodies can stick, and the rest will die due to Crossfire effect—tumor cells distant from the bound antibody can be killed by ionizing radiation from beta-emitting isotopes, important in bulky or poorly vascularized tumors
Basically it is great bc it travels with the circulation and gets to wherever the cancer cells are

Front 28

radiolabeled antibody protocol

Back 28

imaging dose- rituximab followed by ibritumomab-In
therapeutic dose-rituximab followed by ibritumomab-Y

Front 29

Peripheral T-cell Lymphoma (PTCL)

Back 29

-very rare
-very aggressive
-the most common type is Peripheral T-cell lymphoma

Front 30

besides peripheral T-cell lymphoma, what else is a common T-cell?

Back 30

Anaplastic large cell lymphoma ALK+ and ALK-. + is the only T-cell lymphoma with a very good prog.

Front 31

CTCL

Back 31

an indolent subset of PTCL. primarily localized to the skin.
Ex) mycosis fungoides, sezary syndrome

Front 32

unfortunately, most PTCL patients present with

Back 32

symptoms, advanced stage.
disseminated, B symptoms, bone marrow positive, skin lesions. (also use the IPI system)

Front 33

Treatment PTCL

Back 33

Front line: CHOP or Hyper-CVAD (There is no standard of care at this point)
Autologous Transplant for patients in first remission (No Phase III data)
Recurrent disease: Pralatrexate is the first and only approved agent as of 1/2010.
Many agents have been tested