Lymphoma Flashcards
B symptoms:
Which HL subtype is it more common
B symptoms: drenching night sweat, fever >38C, weight loss >10% in 6 months
2/3rd of Lymphocyte poor (1-5% of HLs) present with B Sx. For the others NOdular sclerosing, mixed 1/3rd, while lymph ruch (best prog) <10%
Key IHC markers for NHL vs. HL, identify acceptions and generalities:
PAX8 is a general B cell marker but may be only weakly + in NL
NHL:
Bcells:
CD: 19,20, 79, PAX8 (CD5 is a Tcell marker that is a poor prognostic sign in DLBCL)
B cells germinal centre markers: BCL-2, BCL-6, CD10
T Cells: CD 2,3, 7,8, Natural Killer CD56
HL:
CD 15, 30 (Occaisionally CD20, except nodular lymphocyte predominant which might be a NHL is CD 19,20
Name the broad categories of lyphomas and the key subtypes in each. Which are assoicated with infection?
HL (R-S or popcorn cells) 10% of all lymphomas
- Nodular sclerosing (Classic) 80% M=F mean age 25 (and 25% EBV related)
- Mixed Cellular 15% (no sclerosis, but diffuse effacement,) assoc w/EBV (75%)
- Lymph depleted <5% (complete effaced, bad prog) EBV and HIV
- Lymph Rich - best prog, diffuse effacement
- Nodular Lymphocyte Predominant - POPCORN CELLS, CD20+ (Ritux) might be a NHL - can transform to DLBCL
NHL (80%, 25% are DLBCL, 20% are Follicular):
(Immature) Precursor B-cell lymphoblastic leukemia/lymphoma.
(Mature)
Low grd B = Follicular
Low grd T = Cutaneous T-Cell
Intermediate grd=
1) Marginal zone = Nodular (25% HepC), Extranodal (MALT, H.Pylori, Chlamydia Psittiti, Burgdorfi skin infection), Splenic.
2) Mantle zone
High Grade = DLBCL, Burkitts
Broad definition of:
HL
Neoplasm arising from Germinal Centre B-cells (GCB)
* Classic diagnostic cells are Reed-Sternberg (RS) cells (however, only account for 1-2% of tumour volume).
The rest being infiltration of lymphocytes, eosinophils and plasma cells
4 sub-types with differing bioBeh are defined.
Define a R-S cell:
RS cell (4 things): binucleated with 2 prominent nucleoli, well-demarcated nuclear membrane,
perinuclear halo, and eosinophilic cytoplasm
o Thought to secrete numerous cytokines, leading to B symptoms
DDX for antero-superior mediastinal mass
4T: Thymus, Thyroid, thoracic aorta, ‘terrible’ lymphoma (Hodgkin’s, non-Hodgkin’s lymphoma i.e primary mediastinal B-cell), teratoma (germ cell tumour)
Grading of follicular lymphoma is based on?
Subtypes of germinal centre neoplasm:
Centroblasts (centroblasts large non-cleaved)
More diffentiate centrocytes (smaller cleaved cells)
Grade is based on centroblasts/HPF
Grd 1 <6
Grd III (incurable) >15
Key Germinal centre lymphomas (and markers):
Flollicular and B Cell
CD 10, B-CL 2, BCL 6
Staging of Lymphomas (not risk groups) is based on:
Lugano = Ann-arbor + Cotswold (notable E, X, A/B, RS)
A-a: Stg 1 = one LN region (e.g. Mediastinum), or lymphoid structure.
II - 2 sites ON SAME SIDE of the Phragm
III- Both Sides
IV Diffuse involvement of 1 or more extra-nodal organs or tissues (i.e beyond E which is proximal or contiguous extension).
E= extranodal contiguous extension, e encompassed within an irradiated field
*bulky disease = single nodal mass 10cm or 1/3 transthoracic diameter
RS stage at the time of relapse
Pathological stage at a given site is denoted by a subscript (e.g. M=bone marrow, H=liver, L=lung, O=bone, P= pleura, and D=skin)
Describe some key HL associated Sx:
SYMPTOMS
* Painless lymphadenopathy most common
* B symptoms: drenching night sweat, fever >38C, weight loss >10% in 6 months
* Pel-Ebstein fever: cyclical spiking fevers up to 40C last ~1week then remit for ~1 week due to cytokine release
* Generalized pruritis/ alcohol-induced pain in infiltrated tissues
* Visceral involvement is most frequently spleen – correlation between burden of splenic disease and likelihood
of haematogenous spread
* Marrow and liver involvement occurs almost exclusively in the setting of splenic disease
When is BMAT not required in Lymphoma workup?
Besides the risk group criteria, blood work should include
When PET shows only one site of HL.
BetaHCG (if female….)
CRP - prognostic in HL
HepB/C, HIV - Causitive (Hep C - 25% nodular marginal), HIV (e.g. Lymph depleted), treatment - Ritux can reactivate Hep B
Key HL RFs:
1) EBV - Classic Nodular sclerosing (25%), Mixed (75%)
2) Immunesuppresion
3) 1% of indivuals w/HL have fam Hx
Key risk stratifying systems in Lymphoma
IPI - DLBCL and advanced follicular = “LAKES” LDH>ULN, AG>60, KPS>70, Extra nodel, Stg 3
(IPI>1 and nonbulky = 6 cycles R-Chop, noRT)
FLIPI-2 - I/II Folicular = B-BLAH, where B = Beta-2 microglobulin, H=Hb<120
(Grd and stage used to determine Tx)
R-ISS - Staging Myleoma “BLAK”, Beta2-microgolbulin, LDH, Albumin, Cytogenetic fuck ups (e.g t(14,16))
EORTC - For HL early to determine favourable (no risk factors) from unfavourable (1 or more): “A-MEEN” Age>50, large Mediastinal mass, ESR, no Extranodal, <4 Nodal areas.
(if Unfav 4x AVBP then 30Gy ISRT, if Fav 2xABVD then 20Gy)
Define TLI and IFRT:
Extended field radiation therapy/ total lymphoid irradiation (TLI) – historical/ no longer use
- Large ‘mantle field’ to treat LN in upper part of body + ‘inverted Y field’ to treat LN in abdomen/ pelvis/ groin
- TLI excluding pelvis = sub-total lymphoid irradiation (STLI)
Involved field radiation therapy (IFRT) – no longer used
- Treatment of a LN region (not individual LN) to cover all subclinical disease
- Field is defined by anatomical boundaries
- Most common involved fields are: neck (unilateral), mediastinum (including hilar bilaterally), axilla (including supra and infraclavicular LN), spleen, para-aortic, and inguinal (including femoral and iliac LN)
DEFINE ISRT and INRT:
Involved node radiation therapy (INRT)
- Introduced by EORTC in 2006 for early stage HL
- Smallest effective volume to include only disease sites evident at diagnosis (does not include subclinical
disease i.e. systemic therapy to manage subclinical disease)
- Requires 2 key considerations:
1. Optimal imaging for accurate pre-treatment disease localisation (e.g. with FDG-PET)
2. Precise co-registration of baseline/ pre-chemotherapy imaging (FDG PET) with planning CT for RT
Involved Site radiation therapy (ISRT)
- Introduced by ILROG in 2014
- Slightly larger volume than INRT to allow for uncertainties with INRT (or situations where patients are treated with RT alone)
