CME INDIA Presentation by Dr. Avinash Kumar Singh, DM (Hematology), Consultant haematologist and BMT physician, Paras HMRI hospital and Patna Haematology clinic, Patna, Bihar.

Based on a talk at APICON-2022, Jaipur.

What You Must Know About Lymphoma?

Cancer Prediction is Frightening

What You Must Know About Lymphoma?
What You Must Know About Lymphoma?
What You Must Know About Lymphoma?

What is Lymphoma?

  • Lymphoma is a group of hematological malignancy arising from lymphocytes. Broadly they are divided into Non-Hodgkin’s lymphoma and Hodgkin’s lymphoma.
  • Depending upon cell of origin lymphoma can be divided in B cell, T cell or NK/T cell lymphoma.
  • Depending upon aggressiveness, lymphoma can be divided in to indolent, aggressive and highly aggressive lymphoma.

Know about Primary and Secondary Lymphoid Organs

What You Must Know About Lymphoma?

What are the Risk Factors?

  1. Male Sex
  2. Late middle-aged and older persons with a median age at diagnosis of approximately 65 years.
  3. Body mass index,
  4. Tobacco and Alcohol use, and inflammatory states.
Risk factors for lymphoma include:
Age
Family history
Certain infections
A lowered immune system
Autoimmune conditions
Other factors.

Causes of lymphoma

  • In most cases, there is no known cause for lymphoma.
  • For or a few types of lymphoma, scientists have identified a cause.
  • Most cases of gastric MALT lymphoma are caused by a common bacterial infection called Helicobacter pylori
  • Almost all people who get gastric MALT lymphoma have Helicobacter pylori infection.
  • Breast implant-associated anaplastic large cell lymphoma (BIA-ALCL) is caused by having textured breast implants.
  • Most people with breast implants do not develop BIA-ALCL – but everyone who develops BIA-ALCL has breast implants.

How Lymphoma develops?

  • Origin of Non-Hodgkin B cell lymphomas and mechanisms related to their development During development B cells can acquire translocations, deletions or mutations that make a subtype of lymphoma.
  • Pre-germinal centre derived lymphomas are CLL unmutated and mantle cell lymphoma and some follicular lymphomas.
  • Germinal centre derived lymphomas are derived by transformation from either variable region gene recombination (BCL-2-IgH) in follicular lymphoma, somatic hypermutation (BCL-6) in diffuse large B cell lymphoma, or class switching in c-myc sporadic Burkitt ́s lymphoma.
  • Post-germinal centre B cell lymphomas are marginal zone lymphoma, small lymphocytic lymphoma/chronic lymphocytic leukaemia and plasmacytoma and are derived from memory B cells and plasma cells. 
  • B-Cell Lymphoma: A hallmark of many types of B-cell lymphoma is reciprocal chromosomal translocations involving one of the immunoglobulin loci and a proto-oncogene. As a consequence of such translocations, the oncogene comes under the control of an active immunoglobulin locus, causing deregulated, constitutive expression of the translocated gene.

Presentations

  • Asymptomatic
  • Symptomatic
    • Non-responding fever
    • B symptoms
    • Pain/breathlessness
    • Persistent painless node enlargement
    • Skin rash
    • Can involve any organ from head to toe

Difference between NHL and Hodgkins

Non Hodgkin’sHodgkin’s
COMMON AGEADULTS AND ELDERLYBIMODAL
NODE PATTERNNON-CONTIGUOUSCONTIGUOUS
NODE CONSISTENCYFIRMRUBBERY
PEL EBSTEIN FEVERNOMAY HAVE
RELATION WITH ALCOHOLNOPAINFUL NODES
INCIDENCEMORE COMMONRELATIVELY LESS COMMON

Diagnosis and Classification

  • FNAC is not sufficient
  • Don’t use steroid in suspected case of lymphoma
  • Urgent referral should be done in cases of aggressive or very aggressive lymphoma
  • BIOPSY IS MUST to know D/D

KOCHS/BENIGN LPD/INDOLENT/HODGKINS/METASTASIS/NON-SPECIFIC/VIRAL

What You Must Know About Lymphoma?
  • IHC is must: MCL/FL/PTCL/PBL/HL
  • Molecular Tests are Must: DHL/THL/TARGETS
What You Must Know About Lymphoma?
What You Must Know About Lymphoma?

Courtesy: Management of Lymphomas: Consensus Document 2018 by an Indian Expert Group Indian J Hematol Blood Transfus (July-Sept 2018) 34(3):398–421 https://doi.org/10.1007/s12288-018-0991-4

Staging

What You Must Know About Lymphoma?

Management

What You Must Know About Lymphoma?
  • Performance status – ECOG/KARNOFSKI
  • Co morbidity
  • Emergencies –TLS/SVC/Pericardial tamponade/Luminal obstruction
  • Counselling
  • Consent before chemotherapy
  • Definitive Treatment – as Per Type of Lymphoma – NCCN Guideline
  • Chemotherapy –CHOP/MOPP/ABVD etc.
  • Chemo Immunotherapy-RCHOP/BR/ABVD etc.
  • Immunotherapy-MABS/CPI
  • Cellular Therapy – HSCT/CAR T Cell Therapy

Survival Trend DLBCL

What You Must Know About Lymphoma?

See a Case

  • 30 years old male presented with infradiaphragmatic multiple lymphadenopathy with fever and weight loss for 1 month
  • Work up confirmed as DLBCLL stage IIi non-bulky R IPI good
  • What treatment options?
What You Must Know About Lymphoma?
  • Received 4 cycles of RCHOP
  • iPET CT –CR
  • 2 more R
  • Follow Up
  • In Remission for Last 4 Years
What You Must Know About Lymphoma?

See Case 2

  • 40 yrs. old lady without any co morbidity presented with right cervical persistent painless lad 2X3 CM for 1 month associated with weight loss fever.
  • Work up confirmed it as classical Hodgkin’s lymphoma mixed cellularity type stage 1A Favourable
  • What Treatment Options?
What You Must Know About Lymphoma?
  • Received 2 Cycle ABVD
  • IPET –CR
  • Received 2 More Cycle Of ABVD
  • Follow Up
  • In Remission for Last 6 Years

Relapse/Refractory

  • Case 1 Of DLBCL Relapsed After 4 Years
  • What Options???
What You Must Know About Lymphoma?
What You Must Know About Lymphoma?

What is new in lymphoma management?

Engineering Patients’ Immune Cells to Treat Their Cancers

  • A type of treatment in which a patient’s T cells (a type of immune system cell) are changed in the laboratory so they will attack cancer cells.
  • T cells are taken from a patient’s blood.
  • Then the gene for a special receptor that binds to a certain protein on the patient’s cancer cells is added to the T cells in the laboratory.
  • The special receptor is called a chimeric antigen receptor (CAR).
  • Large numbers of the CAR T cells are grown in the laboratory and given to the patient by infusion. CAR T-cell therapy is used to treat certain blood cancers, and it is being studied in the treatment of other types of cancer.
What You Must Know About Lymphoma?

Final Points

  • Lymphoma is a common hematological malignancy encountered in day-to-day practice.
  • Awareness is the key to detect cases on time.
  • Signs and symptoms are mostly nonspecific and difficult to differentiate from infections.
  • For diagnosis tissue biopsy/IHC is must and molecular tests too are important
  • PET CT is important for staging and response assessment.
  • With better understanding of tumor biology, newer targets and therapy survival has improved.
  • Relapse refractory disease is a challenge and most of the time ASCT is needed to consolidate.

References:

  1. https://www.cancer.net/cancer-types/lymphoma-non-hodgkin/risk-factors
  2. Management of Lymphomas: Consensus Document 2018 by an Indian Expert Group Indian J Hematol Blood Transfus (July-Sept 2018) 34(3):398–421 https://doi.org/10.1007/s12288-018-0991-4
  3. https://www.cancer.gov/publications/dictionaries/cancer-terms/def/car-t-cell-therapy
  4. Brentjens R, et al. “Driving CAR T cells forward.” Nat Rev Clin Oncol. 2016 13, 370–383.
  5. https://www.researchgate.net/figure/Origin-of-Non-Hodgkin-B-cell-lymphomas-and-mechanisms-related-to-their-development-During_fig3_221929542
  6. https://www.nature.com/articles/nrc1589



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