Subtypes of Large BCLs
- LBCL with genetic lesions
- Site Specific
- NOS
Subtypes of Large BCL with genetic lesions
4
- Double Hit (MYC & BCL2) DLBCL
- LBCL with IRF4 (young, GC markers and MUM1)
- HGBCL with 11q abberstions (BL-like morphology, B markers with CD10+, BCL6+, BCL2- and MYC-)
- ALK+ LBCL (immunoblastic, aggressive with CD3+ and CD30+)
What are the site specific Large BCL
11
- 1’ LBCL of immune priveileged sites
- PBL(#)
- EBV+ DLBCL(#)
- 1’ mediastinal LBCL
- Mediastinal Grey Zone lymphoma
- Fibrin associated LBCL(#)
- Lymphomatoid granulomatosis(#)
- DLBCL ass. with chronic inflammation(#)
- Fluid overload LBCL
- Intravascular LBCL
- 1’ cutaneous DLBCL leg type
(#) = EBV associated
Subtypes of Large BCL, NOS
3
I. DLBCL, NOS
* GCB and Non-GCB
* Morphological variants
* Immunoblastic
* Centroblastic
* Anaplastic
II. HGBCL, NOS
* and 11q abberations (BL-like)
III. TCHR BCL
Features of TCHR LBCL
- Large Bs in the background of Ts and Histiocytes (CD68 and CD163+)
- Relationship with NLP HL
- IMPT: Positive for PanB, BCL6. Variable BCL2, MUM1 and EMA. Negative for CD15, CD30, CD138 and EBV-
Subtypes of BL
I. EBV+ BL
* > somatic hypermutation
* < driver mutations
* < BCR expression
II. EBV- BL
* inverse of EBV+ BL
Features of BL
- t(8;14), t(8;22), t(2;8) (MYC to IgH/K/L gene promoter)
- Medium, prominent nucleoli, lipid vacuoles
- Starry sky - tingible body macrophages
- IMPT: Positive for PanB, CD10, BCL6, CD43 (aberrant) and > Ki67 (>95%)
- Tx: R-CODOXM + IVAC or R-Da-EPOCH or HyperCVAD
Hanz Classifier
CD10+ = GCB
CD10-, BCL6+ and MUM1- = GCB
CD10-, BCL6- = Non GCB
CD10-, BCL6+ and MUM1+ = Non GCB
Prognosis of LBCL
IPI (APLES)
IPI:
* Age >60
* Performance Status
* LDH >
* Extranodal sites
* Stage
ECOG:
0 - Fully active
1 - Light work
2 - Self care, no work
3 - Completely disabled
IPI Age Adjusted (<60) Revided IPI (= IPI - predicts outcome of pts on Ritux) NCCN IPI (=IPI - different LDH, sites not nr of extranodal) CNS IPI (=IPI + Renal and adrenal involvement) 4-6 = High risk - CNS prophylaxis (High dose systemic/intrathecal MTX PLUS leucovorin)
CNS prophylaxis in LBCL
CNS IPI
* IPI + Renal and adrenal involvement
* 4-6 = High risk = CNS prophylaxis
* High dose systemic/intrathecal MTX
* PLUS leucovorin
Treatment of LBCL
I. R-CHOP & PET adapted approach
II. Mabs
* CD19 (Tafasitamab)
III. ADC
* CD79a (Polatuzumab)
* CD19
IV. Bispecific Abs
* Glofitamab
