NON HODGKINS LYMPHOMA

PROF OLUSAYO ALAO

MBChB,PGDM,MSc,MBA,FWACP

Non Hodgkins Lymphomas

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14/03/2022

Learning objectives

• Define and classify Lymphomas
• List the differences between HL and NHL
• Discuss etiology of NHL with emphasis on infective agents
• List examples of indolent and aggressive NHL
• State the clinical features of NHL including
• Discuss ‘B’ symptoms of the disease
• Laboratory findings in NHL
• Stage the disease
• Indications for treatment as well prognostic factors

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Outline

• Introduction and classification of lymphomas
• Differences between Hodgkins and non Hodgkins lymphomas
• Non Hodgkins lymphoma

-Epidemiology

-Aetiology

-Classification

-Clinical features

-Laboratory findings

-Staging

-Treatment

-Prognosis

-Conclusion

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Introduction

• Lymphomas are neoplastic disorders of the lymphoid system
• They are heterogenous proliferation and accumulation of malignant lymphocytes in the lymph node and other lymphoid tissues and sometimes in the extra-lymphatic tissues.
• Occasionally, they can spill over into the blood (Leukaemic phase) or infiltrate organs outside lymphoid tissue
• The lymphoma cells (malignant lymphocytes) are derived mainly from B or T- cells and a few from NK cells.

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Introduction

• The tumour cells are often present in the regions of the body with high concentration of lymphoid tissues.
• These include lymph nodes, waldeyer’s ring (tonsil, base of the tongue and nasopharynx), spleen, payers patches and bone marrow.
• Less frequently, the tumour cells can invade extra nodal tissues of the liver, central nervous system (CNS), skin, bone and lungs

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Classification

• Lymphomas are classified into two main types based on the presence or absence of Reed-Sternberg cell
• Hodgkin’s lymphoma (HL)
• Non-Hodgkin’s lymphoma (NHL)
• HL is characterized by the presence of reed-Sternberg cells in a milieu of inflammatory cells
• NHL - Reed-Sternberg cells absent
• NHL more common than HL and has a greater likelihood of dissemination at the time of diagnosis

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Differences btw HL and NHL

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Non Hodgkins’s Lymphoma (NHL)

• These are group of malignant disorders characterized by proliferation of lymphoid cells in lymphoreticular sites including lymph nodes, bone marrow, spleen, liver and GIT.
• They are a heterogeneous group of malignancies that are diverse in cell of origin, morphology, cytogenetic abnormalities, response to treatment and prognosis
• Occasionally some patients may present with abnormal lymphocytes in circulation without significant adenopathy.

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NHL Intro

• Malignant cells comprise about 85% of B cells and 15% of T cells or NK cells
• NHL are generally characterized by irregular pattern of growth and distribution which is often multifocal, centrifugal and non-contiguous.
• Has greater likelihood of dissemination at the time of diagnosis.
• Has greater predilection to spread to extranodal sites than Hodgkin’s lymphoma
• NHL is more common than HD

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Epidemiology

• NHL is slightly more common in males than in females with the ratio of 1.5:1.0
• It is more common in white people than in black people.
• Although NHL can occur at any age, the incidence rises considerably with age. As life expectancy increases there is a consequent increase in incidence of NHL
• Median age of presentation is > 50 years
• High-grade lymphoblastic and Burkitt lymphomas are observed more commonly in children and young adults
• Annual incidence: 5-10 per 100 000 personss

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Why increase in the incidence of NHL

• New diagnostic techniques such as gene rearrangement studies to establish clonality in cases that otherwise would have been diagnosed as benign disorders .
• Better imaging techniques
• Infectious agents/ exposure to environmental hazards
• Improved biopsy / histology techniques
• Ageing population
• Increasing use of immunosuppressive drugs
• Transplantation medicine
• Congenital and acquired immunodeficiency syndromes eg HIV/AIDS

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Aetiology

• No identifiable cause in a large majority of cases
• However several factors are known to play a role
• Chromosomal translocations and molecular rearrangements are implicated
• T(14;18) involving heavy-chain region of the Ig gene on 14 and bcl-2 antiapoptotic oncogene on chromosome 18 respectively, result in over expression of bcl-2 is found in over 85% of follicular lymphomas
• This results in inhibition of apoptosis rather =LYMPHOMA
• Translocation (11;14) results in over expression of cyclin D1 is diagnostic of mantle cell lymphoma.

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Aetiology

• Environmental factors also play a role lymphomagenesis
• Certain workers have a slightly increased risk of NHL eg Farmers; pesticide applicators, flour millers, meat workers, painters, mechanics,
• Workers in the petroleum, rubber, plastics and synthetics industries.
• Some chemicals are linked to the development of NHL eg Pesticides and herbicides (organo phosphates, chlorophenols), solvents and organic chemicals (benzene, carbon tetrachloride) and wood preservatives.
• Patients who receive cancer chemotherapy and/or radiotherapy have increased risk

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Infections

• Chronic Antigenic simulation from viral and bacterial infection has been implicated
• Epstein-Barr Virus (EBV); Endemic (African) Burkitt lymphoma, NHL in immunosuppressed individuals, Post-transplant lymphoproliferative disease (PTLD), Sinonasal lymphoma in Asia and South America
• Human T-cell leukaemia/lymphoma Virus type 1 (HTLV-1); Adult T-cell leukaemia lymphoma (ATLL)
• Human Herpes virus 8 (KSAV); Primary effusion lymphoma (body cavity-based lymphomas ), Lymphomas in patients with HIV infection

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Etiology; Infections

• Hepatitis C Virus; Splenic marginal zone NHL, Lymphoplasmacytoid NHL
• HIV; Primary CNS lymphoma, plasmablastic lymphoma of the oral cavity, primary effusion lymphoma, Diffused Large B Cell Lymphoma (DLBCL),
• H. pylori ; gastric MALT lymphoma
• Borrelia burgdorferri; MALT lymphoma of the skin
• Campylobacter jejuni; MALT lymphoma of the small intestine
• Chlamydia psittaci; MALT lymphoma of the eyes

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Autoimmune disorders

• Sjogren’s syndrome; MALT lymphoma of salivary glands
• Hashimoto thyroditis; MALT lymphoma of thyroid gland

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Aetiology; Immunosuppression

• Congenital immunodeficiency states eg. ataxia telangietasia, wiskott-Aldrich Syndrome, common variable hypogammaglobulinemia, severe combined immunodeficiency
• Acquired immunodeficiency states- HIV infection, iatrogenic immunosuppression in solid organ or bone marrow transplant recipients, patients who receive cancer chemotherapy and/or radiation therapy
• Connective tissue disorders; rheumatoid arthritis, systemic lupus erythematosis (SLE).

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Pathophysiology

• More than 80% of NHLs arise from B cells while the rest from T cells or NK cells
• Either precursor or mature cells may be involved.
• There exist overlap between leukaemia and NHL- both involve proliferation of lymphocytes or their precursors.
• A leukaemia-like picture with peripheral lymphocytosis and BM involvement may be seen in 50% of children and in about 20% of adults with some types of NHL.
• Patients with more extensive nodal involvement (especially mediastinal), fewer circulating abnormal cells and fewer blast forms in the BM (<20%) are considered to have lymphoma.

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Classification

• Classification of NHL has continued to evolve over time

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Classification; biological behaviour

• Low-grade NHL; Indolent and slowly progressive, may respond to treatment but difficult to cure
• Follicular lymphoma
• Small lymphocytic lymphoma
• Lymphoplasmacytoid lymphoma (Waldenstrom’s macroglobulinaemia)
• Marginal zone lymphomas
• Mantle cell lymphoma: some cases
• High-grade NHL; Aggressive but often curable
• Diffuse Large cell Lymphoma (B- or T-cell)
• Burkitt Lymphoma, mantle cell lymphoma (some cases)
• Lymphoblastic lymphomas

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Classification; cell of origin

• Lymphomas of B cell origin tend to mimic normal B cells at different stages of development.
• They can be divided into those resembling
1. Precursor (bone marrow) B cells
2. Germinal centre cells
3. Post-germinal centre cells

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• T cell lymphomas resemble T cells in bone marrow or thymus or peripheral mature T cells

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Clinical features

• Most patient are usually 50+, except in Burkitt lymphoma where presentation can be seen in younger age group.
• Commonest presentation is that of organ infiltration characterised by lymphadenopathy and organomegaly
• Lymphadenopathy ; asymmetric, painless, firm, rubbery, discrete or matted.
• Lymphadenopathy may be generalized at presentation.
• Hepatosplenomegaly is frequent at presentation

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Clinical features

• Symptoms of hypermetabolic state– unexplained fevers, (38’C), drenching night sweats and weight loss (>10% of total body weight in preceding 6 months).These are called B symptoms of a lymphoma
• Symptoms of cytopaenias from bone marrow suppression and/or infiltration.
• Anaemia; tiredness, exertional dyspnoea, features AHF
• Leucopenia; fever, mucositis, cough etc
• Thrombocytopenia; mucocutaneous bleeding

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Clinical features

• Other symptoms and signs may be due to involvement of specific organs e.g. skin, GI tract, lungs, CNS etc
• Waldeyer’s ring involvement; sore throat, obstructed breathing, shortness of breath, cough.
• GIT; abdominal pain and distension
• Skin involvement especially in Mycosis fungoides and Sezary syndrome
• Brain, testes or thyroid glands may be involved

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Clinical features

• History of prior exposure to radiation and other putative risk factors
• Organ transplantation or HIV may provide diagnostic and prognostic insight in cases of NHL.

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Examination findings

• There may be high fever, palor, jaundice, tachycardia and respiratory distress.
• Purpura, petechae or ecchymosis
• Examine all lymph node bearing areas as well as assess hepatomegaly and splenomegaly.
• Pharyngeal involvement, a thyroid mass, pleural effusion, abdominal mass, testicular mass and cutaneous lesions are findings that might direct further investigations and subsequent therapy.
• A neurological examination is vital at diagnosis.

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Examination findings

• Some patients with Waldeyer ring involvement also have involvement of the gastrointestinal tract and vice versa especially in mantle cell lymphoma.
• Patients with paranasal sinus involvement, testicular involvement and epidural lymphoma are particularly prone to have meningeal involvement and thus requires a diagnostic lumbar puncture.
• Patients with one testicle involved are likely to relapse in the contralateral testis.

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Laboratory findings

• FBC; cytopenias if BM is involved or suppressed
• PBF; may show lymphoma cells
• Coombs test may be positive
• Histology of LN and/or BM is key to diagnosis
• BM involvement is found more frequently in low grade malignant lymphomas.
• Normal lymph node architecture is distorted and replaced with neoplastic lymphoid cells
• Pattern of organ infiltration can be diffuse, nodular or mixed
• FNAC; not very useful in diagnosis of lymphoma except in Burkitt lymphoma

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Laboratory findings

• Chemistries including EUCr, LFT, Ca, PO4
• Serology; RVST, HBsAg, HCV
• LDH; levels are raised in rapidly proliferating and extensive disease
• Serum uric acid may be raised.
• SPE (SERUM PROTEIN ELECTROPHORESIS) may reveal a paraprotein
• Urinalysis

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Imaging studies

• CXR; may show mediastinal adenopathy, pleural effusions or pericardial effusions and parenchymal involvement.
• CT-Scan, MRI, Abdominopelvic /scrotal ultrasound scan: may reveal foci of lymphoma
• Endoscopy and Barium studies for GIT lesions
• Gallium scans and position emission tomography (PET) scan provide functional rather than just anatomic information on the foci of lymphoma
• MRI is useful in identifying bone and CNS involvement.
• Molecular Studies; cytogenetics, immunophenotyping, PCR

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Immunophenotypic markers of major subtypes of lymphomas

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Staging; Ann Arbor staging with cotswold modification

• Same as in HL

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Modifying features

A : No systemic symptoms

B : Systemic symptoms- unexplained fever (>38.C) and/or night sweats, and/or unexplained weight loss of >10% of body weight in the previous 6 months

X : Bulky disease

• Mediastinal mass > 1/3 the maximum width of the chest, and/or
• Lymph node disease with maximum dimension > 10 cm

E: Extranodal involvement

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Specific Disease Groups

B CELL LYMPHOMAS

• Follicular Lymphoma
• Burkitt Lymphoma
• Small lymphocytic Lymphoma
• Mantle cell Lymphoma
• Marginal zone B Cell Lymphoma
• Diffuse Large B- Cell Lymphomas
• Lymphoplasmacytic Lymphoma

T CELL AND NK CELL NHL

• Extranodal T or NK lymphoma
• Enteropathy-associated T-cell lymphoma
• Hepatosplenic T-cell lymphoma
• Subcutaneous panniculitis-like T-cell lymphoma
• Cutaneous T-cell lymphoma (Sezary syndrome and mycosis fungoides)
• Primary cutaneous gamma-delta T-cell lymphoma
• Anaplastic large-cell lymphoma
• Angioimmunoblastic T-cell lymphoma
• Primary T-cell lymphoma unspecified

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Burkitt Lymphoma

• Burkitt's lymphoma was first described in 1958 by Denis Burkitt, a surgeon working in Central Africa, Kampala.
• It is an aggressive solid tumor of B Lymphocytes
• The most common childhood tumour in tropical Africa and is one of the fastest-growing neoplasms known, with a tumour doubling time of between 24 and 48 hours.

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Burkitt Lymphoma

• BL is a highly aggressive lymphoma characterized histologically by a diffuse monotonous medium sized cells with a high mitotic rate
• The disease typically involve the maxilla, mandible and abdominal viscera, it can arise wherever B-lymphocytes are found and this explains varied sites of presentation and clinical features.

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Burkitts cell

• The malignant cell is a medium sized cell
• It has a very deep basophilic cytoplasm with numerous lipid vacuoles
• Nucleus with multiple (1-5) basophilic paracentrally situated nucleoli
• Many mitotic figures and apoptotic bodies are present
• These cells are found with numerous benign macrophages giving it the “starry sky” appearance
• The sky= monotonous sheet of malignant cells/lymphoblasts
• The stars= scattered tingible body macrophages (macrophages engulfing dying cells are called tingible body macrophages)

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Epidemiology

• Endemic (African) BL is seen in areas with chronic malaria exposure and is associated with EBV infection.
• The distribution of BL seems to be influenced by some climate factors. High incidence is noted in areas with altitudes usually< 1500m, annual rainfall not less than 50cm and ambient temperature not below 26.6^o c
• In tropical Africa 90% of patients present btw ages 4 & 9years with peak age of 5yrs.
• No cases have been reported before the age of 1yr and rarely above the 20 yrs.
• Male to female ratio is 2:1

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Aetiology

• The cause of BL is not known
• However there is strong association between Epstein-Barr virus (EBV) and BL. Evidences include
• In 1964 EBViral particle was recognised in electron micrograghs of BL cells.
• BL patients have high antibody titres to EBV antigens.
• Over 80% of the tumours contain multiple copies of EBV-DNA genome
• Over 80% of African children who had infectious mononucleosis went on to develop BL.
• Malaria has been implicated as a cofactor in the aetiology of BL

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Classification

• Endemic form of BL
• Largely found in Africa
• characteristically affects facial skeleton
• 4-9yrs with peak age of 5yrs
• Sporadic form of BL
• Occurs anywhere in the world
• Affects mainly Abdominal viscera
• Can occur at any age
• Immunodeficient-associated BL: seen in immunodeficient PATIENTS :HIV/AIDS, organ transplantation patients

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Pathophysiology

• In virtually all cases there is translocation involving c-myc oncongene on one hand and genes that code for immunoglobulins on the other hand.
• The c-myc on chromosome 8 is translocated to gene for Ig heavy chain on chromosome 14 t(8;14), or to gene for kappa light chain on chromosome 2 t(2;8) or lambda light chain on chromosome 22 t(8;22)
• This translocation leads to an over expression c-myc oncogene which is involved in cell proliferation and control of apoptosis
• The gene is also expressed in parts of the cell cycle at a time it should normally be switched off
• This leads to uncontrolled proliferation of B-lymphocytes

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Presentation

• Jaw swelling is the commonest presentation
• May be unilateral or bilateral involving maxilla and mandible.
• Swelling is painless, fast growing and of short duration
• There may be protrusion of the eyes if maxilla is involved
• Gingival swelling, weakness and loose of the teeth at early age
• Abdominal swelling from ovarian, renal, mesenteric masses.
• There may be vomiting or constipation
• May be weight loss, Fever, drenching night sweat
• Neurological deficit if CNS is involved.

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Presentation

• Physical examination findings depend on the sites involved
• Jaw swelling: hard, irregular, non tender.
• Protrusion of the eye balls if there is maxillary swelling.
• Gingival swelling could result in loose teeth and dental anachy.
• Abdominal swelling with intraabdominal masses described as firm and non tender.
• Painless lymphadenopathy esp cervical nodes
• Examine for thyroid, testicular and ovarian masses which are occasionally present.

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Dental Anarchy

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Diagnosis

• Histology shows diffuse infiltrate of medium lymphoid cells, with round or oval nuclei, with coarse chromatin, several nucleoli and abundant basophilic cytoplasm
• Numerous apoptotic bodies surrounded by macrophages that engulf them giving characteristic starry sky ‘appearance’
• Immunophenotyping; positive for CD10, CD19, CD20, BCL6; negative or weakly positive for BCL2
• Cytogenetics reveals t(8;14), (2;8) and (8;22)

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Staging

• STAGE A ; Single extra-abdominal tumor
• STAGE AR; Intra-abdominal tumor >90% surgically resectable
• STAGE B; Multiple extra-abdominal tumors, excluding bone marrow and/or CNS disease
• STAGE C; Unresectable abdominal tumours without bone marrow and CNS disease, but regardless of other sites
• STAGE D; Any of the above with bone marrow and/or CNS disease

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Diffuse large B cell lymphoma

• A heterogeneous disorder representing the classic high grade lymphomas
• The most common NHL world wide representing 25-30% of all adult NHLs
• Diffuse growth pattern, highly invasive and extranodal involvement is common
• Presents have rapidly growing lymphadenopathy associated with a fast rate of cellular proliferation
• Progressive infiltration of bone marrow, GIT, brain, spinal cord, kidneys
• Curable in ~ 40 -50% but can be fatal if left untreated

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Diffuse large B cell lymphoma

• Biopsy shows large tumour cells with prominent nucleoli.
• They are subdivided into ‘germinal centre’ and ‘activated B cell’
• Immunophenotype, CD19, CD20, CD22, CD79a, Frequent CD10
• Cytogenetics; t(14;18) and t(3;4)

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Gross and Histology of LN

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Follicular Lymphoma (FL)

• the commonest indolent (low grade) NHL
• It is characterized by a benign course for many years.
• The median survival from diagnosis is approximately 10 years.
• Patients are likely to be middle-aged or elderly with median age of onset of 60 years.
• Hallmark of FL is translocation involving IgH gene on Chromosome 14 and the bcl-2 gene on Chromosome 18
• This leads to constitutive expression of the BCL-2 gene with increased survival of cells and reduced apoptosis.

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Follicular Lymphoma (FL)

• Patient usually present with painless lymphadenopathy often generalized
• The histological appearances are graded as I–III according to the relative proportion of centrocytes (small cleaved cells) and centroblasts (large uncleaved cells).
• Both grades 1 & 2 are indolent disease
• Grade IIIb patients have the worst prognosis and are generally treated like diffuse large B-cell lymphoma
• Bone marrow involvement is frequent.
• The cells are typically CD10, CD19, CD20, BCL2 and BCL6 positive

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Mantle cell lymphoma

• Commonly involve lymph nodes, but can involve extranodal sites
• Accounts for 1 in 20 of all cases of NHL
• Most patients are more than 50 yrs of age, with M:F of 3:1
• Clinical presentation is typically with lymphadenopathy and often there is bone marrow infiltration.
• Malignant cells are small to medium lymphoid cells
• A translocation t(11;14) juxtaposes the cyclinD1 gene on chromosome 11 to the Ig heavy-chain gene
• This leads to increased expression of cyclin D1
• Immunophenotyping; CD19+, CD20+ CD5+, CD22+ and CD23−
• Cytogenetics t(11;14)

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Small lymphocytic lymphoma

• SLL is low grade lymphoma and always of B cell type.
• This term is used for cases with the same morphology and immunophenotypic markers as B-cell chronic lymphocytic leukaemia (CLL)
• However peripheral blood B lymphocytes is less than 5 × 109/L
• SLL present with lymphadenopathy and/or splenomegaly due to infiltrating CLL type cells
• Typically middle-aged to elderly
• Treatment is as for patients with B-CLL.

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Indications for treatment of NHL

• Rapid increase in node size
• Large tumour load
• Constitutional symptoms
• Advanced disease (stages III and IV)
• Compromised organ functions like pressure effects on CNS
• Tumour related myelosuppression
• Auto immune haemolytic anaemia

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Before initiating therapy

• Confirm histologic diagnosis
• Establish tumour burden (disease staging)
• Confirm tumour immunophenotype
• Confirm tumour cytogenetics
• Counsel Patients/Relations
• Nature of the disease
• Therapeutic options and costs
• Complications of therapy
• Disease prognosis
• Prevent and prepare for management of complications; TLS, Infertility etc

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Treatment

• Treatment options vary due of the heterogeneity of NHL
• Therapeutic decisions are influenced by the patient’s age, performance status, stage of the disease and histological findings
• Indolent Vs Aggressive histology.
• There is no advantage for immediate chemotherapy in indolent lymphomas, watch and wait approach is recommended for asymptomatic disease.

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Algorithm of treatment of NHL

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Treatment of Indolent NHL

• Stages 1 & 11 watchful waiting in early disease is recommended
• Generally treatment is with the use of chemotherapy, Radiotherapy, targeted therapy and High-dose therapy (HDT)
• Single agent therapy with Alkylating agents +/- prednisolone eg Chlorambucil, Cyclophosphamide +/- Prednisolone
• Combination chemotherapy can be used with various combinations eg CVP, COPP, CHOP, CEOP
• Rituximab may be added to the therapy for B cell NHL

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Aggressive disease

• Combination chemotherapy is mandatory
• Radiotherapy may be needed to relieve bulky disease or large extra nodal disease
• CHOP is the gold standard for both B and T-cell disease
• Lymphoblastic lymphomas are treated like acute lymphoblastic leukaemias with COAP
• Burkitts lymphoma is with R-CHOP
• CNS disease is treated with Methrotrexate, Cytarabin or Hydrocortisone

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Prognosis

• Adverse prognostic factors in NHL:
• Age > 60 years at presentation
• Presence of constitutional “B” symptoms
• Elevated serum LDH
• Poor performance status
• Elevated uric acid
• High proliferative rate based on Ki-67 value
• Advanced Stage III or IV disease,
• Bulky tumour (eg diameter > 10cm),
• Presence of 2 or more extranodal sites

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Conclusion

• Non- Hodgkin Lymphomas are heterogenous chronic lymphoid malignancies
• The disease distribution is often mulitifocal, centrifugal and non contiguous
• About 70-85% are of B cell origin while the rest are derived from T lymphocytes or NK cells
• Natural history and clinical presentations are variable
• Biologically they are generally divided into indolent and aggressive lymphomas
• Prompt diagnosis and treatment is key to improve the quality of life of our patients

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The end

• Thank you for listening!

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