12-1: 1/13 - Non-Hodgkin's Lymphoma Flash Cards

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Title:	12-1: 1/13 - Non-Hodgkin's Lymphoma
Description:	Dumenco
Number of Cards:	37
Save Count:	0
Author:	bryant.faria9
Created:	2012-01-18
Tags:	heme
Private	No

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- Question
- Answer
- Side 3

- 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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