Mantle cell lymphoma Flashcards
Tx goals
Usually not curative
Mainly to alleviate Sx
OS with Tx
8-10 years
Subset with indolent disease
Clinically: lymphocytosis, a few enlarged lymph nodes, and splenomegaly
SOX11 negativity
Ki-67 <30%
Evaluation
HBV, HIV BMB if cytopenias LP if blastic variant PETCT Endoscopies if Sx Echo Fertility preservation
Tx of young fit
age < 60, ECOG <2
BR / R-CHOP / Hyper- CVAD
followed by autologous HSCT
followed by R maintenance
Tx of pts > 60
If fit and HSCT candidates- same as younger
If unfit BR and R maintenance
R-Hyper-CVAD
Addition of cytarabine and methotrexate
Two courses A with CVAD B with only Cytarabine and Methotrexate
Better PFS but unacceptable toxicity
R-DHAP
R
Dexamethasone
High dose Ara-C (cytarabine)
Platinol (Cisplatin)
Usually alternating R-CHOP/R-DHAP
M:F
3:1
Median age
68
Types
Classic- nodal, Extranodal (GI)- SOX11 positive
Leukemic- Peripheral blood, BM, spleen, spares lymph nodes (more indolent)- SOX11 negative
Advanced disease %
70-80%
B symptoms %
33%
Immunophnotype
Suraface IgM IgD CD19, CD20, CD5 FMC7 SOX11 Cyclin D1 in Cyclin D1 negative- Cyclin D2/3 will be positive Cyclin E- aggressive P53 (variable) CD23 negative
Genetic featuresleo
t(11:14)- CCND1/IgH
Trosomy 12- 25%
TP53
Extranodal disease
25%
mainly GI
Waldeyer ring
שקדים, אנאואידים וקשריות תת-לשוניות
Cytologic subtypes
Classic
Blastoid- aggressive
Pleomorphic- aggressive
Bulky disease
25%
high LDH
< 50%
DD
Mainly CLL
CLL vs MCL
CD5+ both CD20- bright, CD23 negative, CD200 negative- MCL CD20-dim, CD23+, CD200+- CLL LEF1+- CLL t(11,14)- MCL
Lymphomatous polyposis
Rare presentation
Extensive polyps in GI tract
Anywhere along the tract
Most common distal ileum and colon
MIPI
Age- 50, 60, 70
ECOG
LDH- 0.67, 1, 1.5
WBC- 6.7K, 10K 15K
Tx of non autologous HSCT cadidtates
BR +/- addition of ibrutinib
