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37 Cards in this Set

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Non-Hodgkin's Lymphoma:
Age distribution
Increases exponentially with age esp after age 40
What is the distribution of HL and NHL in children?

What do the NHLs have in common?
40% lymphomas in kids are HL

20% Large Cell Lymphoma)

20% Burkitts

20% Lymphoblastic

ALL three of the NHLs are highly aggressive.
What is the most common NHL in children?
Burkitt's
What are the most common NHLs in adults?

Which are indolent?
-Diffuse Large Cell Lymphoma
-Follicular Lymphoma is number 2
-Small Cell Lymphocytic
-Marginal Zone

Follicular, Small Cell, Marginal Zone are INDOLENT (slow growing) in adults
EBV is a risk factor for _______.
Burkitts
HIV-related lymphomas
NK/T-cell lymphomas (GI Tract, Skin--Asia, Central America)
HTLV-1 is a risk factor for _______.
Adult T-cell Leukemia/Lymphoma (Japan, Caribbean)
H. pylori is a risk factor for _______.
Primary gastric lymphoma (MALT)
HHV-8 is a risk factor for _________.
Body-cavity lymphomas in HIV+
What it Post-Transplant Lymphoproliferative Disease?
100x inc'd risk in NHL in organ transplant immunosuppression

If ummunosuppression is reversed, some will regress spontaneously!
Clinical features of NHL (general).
Asymmetric painless LAD; may be multiple LNs, peripheral Nodes.

Non-contiguous spread.

EXTRANODAL INVOLVEMENT
What is extranodal involvement?
Involvement of BM (anemia, leukopenia, thrombocytopenia)

GI tract (acute abdomen)

Waldeyer's ring (sore throat, noisy breathing); picked up via Head/Neck CT

Skin, testis, CNS, thyroid
Which NHLs have predilection for CNS involvement?

What non-CNS findings increase chances of CNS involvement?
Burkitt's
Lymphoblastic Lymphoma
HIV-related NHL

(NEED CT and CSF)

BM, testis, paranasal/nasopharynx involvement assocd w/CNS involvement!!
What is the greatest predictor of prognosis in NHL?
Histologic subtype
Indolent vs Aggressive Lymphomas (general definitions)
Indolent: slow growing; hard to cure

Aggressive: fast growing; potentially curable (chemo targets cells that multiple fastest)
What cancers are considered Precursor B-Cell neoplasms?
Pre-B lymphoblastic leukemia/lymphoma (pre-B ALL)--occurs in BM. ALL!

This is the only Precursor B-Cell neoplasm. Everything else is mature (peripheral) B cell neoplasm.
What cancer as considered to be Precursor T-Cell neoplasms?
Pre-T lymphoblastic lymphoma/leukemia (pre-T ALL)--occurs in THYMUS.

This is the only Precursor T-Cell neoplasm. Everything else is Mature (Peripiheral) T Cell.
What exam finding is consistent with a Pre-T lymphoblastic leukemia?
Mediastinal mass!

Thymus involved!
NHL tend to be ___-cell.
B-cell
Lymphoblastic lymphoma is an [aggressive/indolent] neoplasm of ____-lymphoblasts.

It is seen more frequently in [adults/children].
Lymphoblastic lymphoma = highly aggressive neoplasm of Pre-T lymphoblasts (mostly Pre-T)

Accounts for 1/3 of childhood lymphomas!
Lymphoblastic Lymphoma:
Typical Presentation
Stage at Presentation
Tdt +/-
Adolescent male w/large mediastinal mass (thymus involved) and pleural effusions.

Life-threatening airway or vascular compression!

Stage IV>70% at dx (extranodal sites)

Tdt+!
In this lymphoma, the gonads and CNS act as sanctuaries.
Lymphoblastic lymphoma

Must include CNS and gonads in tx plan
Which cancers present as Tdt+?
Lymphoblastic Lymphoma (T-Cell)
ALL
Lymphoblastic Lymphoma:
Treatment
Antrhacycline
Steroid
Vincristine-induction

CNS prophylaxis

Prolonged maintenance therapy

Survival rate for children (at 5 years) is 80-90%! Worse prognosis for adults.
What is the most common NHL?
Diffuse Large B Cell Lymphoma; 30% of adult NHLs, 20% of childhood lymphomas.

AGGRESSIVE
Diffuse Large B Cell Lymphoma:
Stage at Presentation
Presentation
Risk Factors for Adverse Prognosis
50% present Stage I/II (early) but rapidly progresses to disseminated disease (IT IS AGGRESSIVE)

Presents with extra-nodal involvement (GI tract, testis, paranasal sinuses)

Risk Factors for poor prognosis:
>60
Stage III/IV
HIGH LDH
2+ extranodal sites
ECOG 2+
This lymphoma exhibits an elevated Ki-67.
Diffuse Large B-Cell Lymphoma

Negative TdT!!!
Diffuse Large B Cell Lymphoma:
Treatment
R-CHOP:
Rituximab (CD-20; B-cell dz)

Cyclophosphamide - alkylating agent
Hydroxydaunorubicin - intercalating agent
Onvocine = VINCRISTINE (MT)
Prednisone

Very high cure rates!
Activated B-Like Gene Expression vs Germinal Center B-like Expression
Activated B-Like Expression = worse prognosis for pts with Diffuse B Cell Lymphoma (means B cells have passed through GC)

Germinal Center B-Like gene expression = better prognosis
Follicular Lymphoma:
Indolent/Aggressive
Presentation
Stage at Presentation
Indolent--10-12 year survival

Presentation: Painless LAD in multiple regions

90% of pts have disseminated disease at presentation (Stage IV)
Indolent lymphomas are not found in ________.
Children
Small-cleaved cells are indicative of _______.
Follicular lymphoma
Follicular Lymphoma:
Extranodal Sites Involved
Histologic Appearance (at nodes at extranodally)
Follic Lymphoma:
BM involvement at diagnosis in 60-70% of pts

BM involvement demonstrates paratrabecular groups of cells (growth of cells near trabeculae of BM)

In LNs, will exhibit FOLLICULAR (nodular) pattern of growth
bcl-2:
Role
Involvement in Cancer
Relevant translocation
bcl-2 = anti-apoptotic protein

Overexpressed in follicular lymphoma in t(14;18)--detected in 85-90% of follicular lymphoma!
Follicular Lymphoma:
Tends to transform to ______
Why does transformation occur?
Grading
Follicular lymphomas can transform into Diffuse Large B Cell Lymphomas (goes from indolent to aggressive)

Transformation is result of acquisition of second mutation

Grade 3 = large number of large cells; treated as thought patients were DLBLC (Grade 3 is on its way to transformation!)
Which cancers employ watchful waiting as a therapy?
CLL
Follicular Lymphoma
Follicular Lymphoma:
Treatment
Initially WW, but if disease gets worse:
Chlorambucil (alkylating agent)
Fludarabine
Rituximab (CD-20)
Bendamustine (alkylating agent!)
Radioimmunoconjugates are indicated for ___________.

2 examples.
Radioimmunoconjugates indicated for relapsed/refractory follicular lymphoma.

Tositumomab
Ipritumomab

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