Lymphomas Flashcards
what percentage of NHL are T and B cell. what percentage high and low grade
85% B cell. 15% T cells
Low grade: 20-40%. high grade: 60-70%
whats the incidence , prognosis, treatment of LB
200/yr. 3rd childhood lymphomas, 3-4% adult.
High grade lymphoma/ mature B-ALL (der14)
intermediate prognosis- good on intensive treatment
Treatment targets proliferating cells: cyclophosphamide, vincristine, methotrexate, etoposide
whats the incidence , prognosis, treatment of DLBCL
4,400/yr. IGH-BCL2 (50%), BCL6 (20-35%), MYC (10%).
BM involvment 11-24%. high grade
Treatment targets Anti-apoptotic cells (40-60% cure)
CHOP-R (rituximab, cyclophosphamide, hydroxydaunorubicin, oncovin, preonisolone)
whats the prognosis, treatment b-cell lymphoma, unclassifiable, with features intermediate between DLBCL and BL. Now called High Grade B-cell lymphoma with MYC and BCL2 and/or BCL6 rearrangements.
double/triple hit.
aggressive, high grade: poor prognosis median survival 4.5-6 months.
cells are proliferating and Anti-apoptotic so DLBCL and BL treatments are ineffective
discuss mantle cell lymphoma
5% NHL, 400/yr.
95% have CCND1-IGH (CCND1: variable bkpts: 50% 8-10kb cluster; 50% over 100kb away)
secondary abns: del(13q), delTP53, delATM, rearrMYC, +3q26, del(9p21)
caused by excessive proliferation before mature and capable of functioning. Aggressive clinical course
Discuss follicular lymphoma
20% NHL (commonest) (60-80% progress to DLBCL)
80% have BCL2-IGH (15% have BCL6)
secondary abns: del (6q21). del(6q23q26) and del(17p) indicate disease transformation.
70% healthy popn have BCL2-IGH
Rituximab (anti-CD20)
Discuss Marginal Zone
extranodal: MALT: BIRC3-MALT1 (20-50%), IGH-MALT1 (20%) (see bcl10-igh, igh-fozp1).
Splenic: del7q, +3, +18
NodalMZL: +3, +18
discuss anaplastic large cell lymphoma
T-cell lymphoma. (3% adult, 10-20% childhood)
NPM1-ALK t(2;5)(p23;q35) (85%)
TPMS-ALK t(1;2)(q25,p23) (15%)
prognosis is better for ALK+ than ALK-. treat with CHOP
Discuss hairy cell leukaemia
2% adult leukaemia; 52yrs; often have dry tap.
BRAF V600F (exon15): increase cell proliferation and survival
Third patients have TP53 mutations also
Discuss Waldenstroms macroglobulinamia
Diagnosed by: presence of monoclonal IgM and LPL (lymphoplasmocytic lymphoma)
Strong ass. Between WM and MYD88 L265P
Separated from MM; MGUS; splenic MZL; MALT lymphoma
Describe the aberrations seen in Burkitt-like lymphoma with 11q aberrations
Proximal gains and telomeric losses of 11q
Lack MYC rearrangement
What mutations are seen in follicular lymphoma
CREBBP, KMT2D (MLL2), EZH2 (20-25%- early therapeutic target)
What mutations are seen in sporadic Burkitt lymphoma
TCF3 and ID3. In 70%
Describe the aberrations seen in Burkitt-like lymphoma with 11q aberrations
Proximal gains and telomeric losses of 11q
Lack MYC rearrangement
What mutations are seen in follicular lymphoma
CREBBP, KMT2D (MLL2), EZH2 (20-25%- early therapeutic target)
