Indolent non Hodgkin lymphomas
Dr. Rajib BhattacharjeeJunior Resident
IPGMER, Kolkata
WHO classification 2008
Most common types of NHL encountered in clinical practice
types of NHL Incidence DLBCL 33%
Follicular Lymphoma 22%
Marginal Zone Lymphoma 10%
PTCL 10%
SLL/CLL 7%
Mantle cell lymphoma 7%
Types of NHL based on aggressiveness
Low grade Intermediate grade high grade
Follicular lymphoma
Marginal zone lymphoma
SLL/CLL
DLBCL
PTCL
Burkitt’s lymphoma
Lymphoblastic lymphoma
New ticket day… in the OPD…
Our patient enters…. A man in his 60s
•Median age at presentation – 55-65 years
•Males are affected more than females
I started to listen to his clinical history..
He complained of a painless swelling in his neck..
2/3rd of NHL patients present with asymptomatic lymph node swelling (nodal disease)Common in FL,MCL & SLLSites- Neck 70%Groin 60%Axilla 50%
Any extranodal disease…
• I asked him,” Do you have any problem during swallowing or do you get full with little food?”
1/3rd of NHL patients may present with extranodal disease.Common in DLBCL & MZLSite-GIT - 25-35%Waldayers ring – 18-23%
B Symptoms
I asked 3 questions:-1. Did you suffer from fever in the last few
months?2. Have you lost a lot of weight lately?3. Do you change your shirt often due to night
sweats?Then I asked… did you find any cause to these or
were they unexplained?
Exam time..
• Lymph node examination• Head and neck – waldeyer’s ring• Chest – Sternal/2nd ICS percussion Pleural effusion• Abdomen- Organomegally, Lump, Ascites Testes• Skin - Nodules
Investigations ..
Lab investigations• CBC, KFT, LFT, Electrolytes• Ca2+
• LDH, Uric acid• HBsAg, IgM HBc• HIV I & II
Imaging• Chest X-Ray• CT Thorax, abdomen & pelvis• PET CT• Tc-99m Bone Scan• MUGA/ Echocardiography
Endoscopy • Upper GI
• Bone marrow biopsy A must for all NHL patients (SLL, mantle cell lymphoma – 70% FL – 50% , DLBCL – 15%)
• CSF Cytology Only in suspected leptomeningeal
involvement
Histopathological examination
The cervical lymph node must go for biopsy….
A medium sized accessible lymph node is preferred for excision.Cervical lymph node if palpable, is preffered
Which stage is the disease..???
X = Bulky disease• Clinically diameter > 10cm• CXR PA- Mediastinal mass ratio(MMR) = Max width of mass__ > 0.33
Max intrathoracic dia = Max width of mass >0.35 Intrathoracic dia @ T5 - T6
Possible histologies for our discussion
Follicular Lymphoma
5Y OS
70.7%
50.9%
35.5%
How do I treat this patient if he has localised FL – Stage I & II?
Gr 1-2, non bulky, asymptomatic
IFRT (30Gy)
Gr 3, bulky, B symptoms
IFRT(30Gy) RCHOP (4 cycles)
Boost (upto 40 Gy) IFRT to the bulky site (30Gy)
How do I treat this patient if he has advanced FL – Stage III & IV?
Asymptomatic
Observation or Rituximab
Symptomatic, B symptoms, cytopenias, compromised end organ function
RCVP/RCHOP (6 cycles)
Gr 3 RCHOP (6 cycles)
Important studies study Conclusion
BNLI Study 2003 Observation is a good initial approach in asymptomatic stage III & IV FL
GLSG Trial 2005 & Marcus et al Rituximab with both CVP & CHOP produce enhanced OS
SWOG Trial Anthracyclins fail to improve OS in indolent lymphoma
Bendamustine in 1st line
indolent/mantle cell
R-B R R-CHOP 69.5mo PFS 31.2mo
toxicity
Maintenance Rituximab
Follicular lymphoma RCHOP/RCVP/RFCM
mRituximab R Obv
75% PFS 58%
72% CR 52%
Progressive disease
• Elderly, asymptomatic – Obv• 2nd line chemotherapy – Bendamustine FCM• Radioimmunotherapy – Zavaline, Baxxar• mTOR inhibitors – NVP-BEZ235• Proteasome inhibitors• Stem cell transplant
Marginal zone lymphoma
Three entities• Nodal • Extranodal - MALToma• Splenic
StomachSmall intestineOccularSkinParotidThyroid
How to treat…Extranodal disease• Symptomatic local tumor – RT• Generalized disease Asymptomatic – Obv Symptomatic - CT (Chlorambucil)
Nodal disease – R-CHOP
Splenic disease – Splenectomy or Splenic RT
Gastric MALToma• C/F- abdominal fullness, loss of appetite,
waight loss, B symptoms, pain, bleeding• Diagnosis- Endoscopic biopsy• H. pylori association in 92% (# Isaacson et al)• Antibiotics and PPI (even in H.pylori –ve cases) CRR-75% 5Y FFS-50% OS>90%• Endoscopy after 3 months• Lymphoma persist- *deep invasion *t(11:18)• Antibiotic failure T/t – RT(24-30Gy)• Not suitable for RT – Rituximab monotherapy
SLL
• Localized disease – IFRT (30Gy)
• Advanced disease – Chemoimmunotherapy
Age>70, co-morbidities Age<70, medically fit Relapse
•Obinutuzumab + Chlorambucil•Rituximab + Chlorambucil•Bendamustine + Rituximab
•FCR (Fludarabine + Cyclofosfamide + Rituximib)
•Ibrutinib
IFRT
Fallow up
• Physical exam & labwork - 3-6 months for 1st 5 years then annually.
• CT scan every 6 months for 2 years then annually.
THANK YOU
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