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Lymphoma 101

Lymphoma 101. Armaan Khalid. What is Lymphoma?. Cancer of the lymph nodes Lymphoma > Leukaemia Commonly manifest by the development of lymphadenopathy at single/multiple sites Classified on basis of histological appearance Hodgkin’s lymphoma (HL) Non-Hodgkin’s lymphoma (NHL).

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Lymphoma 101

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  1. Lymphoma 101 Armaan Khalid

  2. What is Lymphoma? • Cancer of the lymph nodes • Lymphoma > Leukaemia • Commonly manifest by the development of lymphadenopathy at single/multiple sites • Classified on basis of histological appearance • Hodgkin’s lymphoma (HL) • Non-Hodgkin’s lymphoma (NHL)

  3. Hodgkin’s Lymphoma (HL) • Involves primarily the lymph nodes • ♂:♀ is 1.3:1 • Peak incidence is in 3rd decade • Tumour of B Cell origin • Presence of Reed-Sternberg Cells on pathology • Often presents @ a single site & spreads in a predictable fashion

  4. Aetiology of HL • ?Link with EBV • Evidence linking previous infectious mononucleosis with HL • 40% of patients ↑ EBV titres @ time of diagnosis

  5. It’s all about the Reed-Sternberg’s • Hallmark of HL is Reed-Sternberg cells • Large cell w enlarged multilobulated nucleus, prominent nucleoli & abundant eosinophilic cytoplasm • RS cell variants exist in HL subtypes • Staging based on the Ann Arbor Classification • Used to stage both HL & NHL

  6. Classification of HL • Nodular Sclerosing HL (70%) • Typically seen in young adults/adolescents & involving Mediastinal & neck LN • Characteristic cell is the lacunar type RS cells • Overall prognosis is good • Mixed Cellularity HL (25%) • Most common HL in pt > 50y/o, ♂ predominance • Classical RS cells abundant • Assoc with B symptoms • Commonly affects abdominal LN & spleen • Most common HL subtype in HIV +ve population

  7. ‘Classic’ Reed-Sternberg Cells *Common in mixed-cellular subtype HL

  8. ‘Lacunar-Type’ RS Cells *Common in nodular sclerosis subtype HL

  9. Classification of HL • Lymphocyte-rich HL (5%) • Characterised by an inflitrate of many small lymphocytes & RS cells; often an indolent disease • Lymphocyte-depleted HL (1%) • Rare & lacks cellular infiltrate with numerous RS cells • Seen in HL assoc with HIV • Patients usually w advanced stage disease • Nodular Lymphocyte-predominant HL (5%) • Characterised by large number of small resting lymphocytes admixed with benign histiocytes • Classical RS cells difficult to find • Lympho-histiocytic (L&H) variant RS cell (popcorn cell)

  10. Pathogenesis of HL • ??? • Central issue: Lymphocytes of the B-cell lineage not expressing immunoglobulins escape apoptosis • ?Role of EBV • Interferes w normal immune regulatory mechanisms • Often localised to single site & spreads in pedictable fashion

  11. Clinical Features • LN enlargement, esp. Cervical LN • Painless & of rubbery consistency • Supra-diaphragmatic LNs affected in 80% • Hepatosplenomegaly • B symptoms (40%) • Fever, drenching night sweats, weight loss > 10% in 6/12 • Pruritis, fatigue, anorexia, lethargy, malaise • Sg/Sx due to organ involvement • Due to mass effect (Bone, Lung, Cardiac, Gut) • SVC Syndrome • Due to biochemical derangement

  12. Before & After

  13. What is causing the Blockage?

  14. Superior Vena Cava Syndrome • Obstruction of the SVC by an upper mediastinal mass • Sg/Sx • Difficulty breathing/swallowing • Stridor, swollen, oedematous facies • Venous congestion • Ix • Imaging (CXR, CT chest, Invasive contrast venogram) • Rx • Based on what is causing the obstruction • Steroids, diuretics, stenting, chemo/radioRx

  15. Differentials • Infectious Mononucleosis • Lung Ca (Small Cell) • HL • NHL • Rheumatoid Arthritis • Sarcoidosis • SLE

  16. Investigations • FBE • ESR • LFT • Serum LDH (Correlates with ‘bulk’ of disease) • CXR • HIV screening • CT scan • PET scan • Bone marrow aspirate & trephine (BMAT) • LN biopsy

  17. Ann Arbor Staging Classification

  18. Further Designations to Ann Arbor

  19. Management • Rx taken with curative intent • Rx based on: • Anatomical distribution of disease • ‘Bulk’ • Presence/Absence of ‘B’ symptoms

  20. Early Stage (IA, IIA, No Bulk) • Brief chemotherapy + radiotherapy • Example: ABVD or BEACOPP • A = Adriamycin (Doxorubicin) • B = Bleomycin • V = Vinblastine • D = Dacarbazine

  21. Advanced Disease • Cyclical combination chemoRx w or w/o radioRx • Gold standard: ABVD • Pt w bulk disease receive radioRx • Curative 50-60% • Risk of myelosuppression, infertility, 2° malignancy & organ damage

  22. Non-Hodgkin’s Lymphoma (NHL) • Classified separately from HL • 70% - 80% are of B Cell origin • Incidence 15/100 000 per year in devt countries • Slight ♂ predominance • Median age of presentation 55-75y/o • Can be classified on the basis of origin • Tumours of B cells • Tumours of T cells • Tumours of NK cells

  23. Aetiology NHL • Unknown cause; wide geographical variation • ?Envt factors • EBV (Burkitt’s lymphoma) • ↑% of lymphoma in pts with AIDS • H. Pylori in gastric MALT lymphoma

  24. Burkitt Lymphoma (African subtype)

  25. Pathogenesis NHL • Malignant clonal expansion of lymphocytes • Due to errors in gene rearrangements or recombinations • Translocation errors with specific genes • Burkitt’s (c-myc)

  26. Clinical Features • Same as HL* • Same as HL Investigations

  27. Clinical Differences b/w HL & NHL

  28. Follicular B Cell Lymphoma • HOPC Asymptomatic w painless generalised lymphadenopathy • Occurs ↓% in Asian population & predominantly in older people (>20-30y/o) • Ix indicates multiple site involvement (Bone marrow infiltration is common) • Runs a remitting & recurring clinical course • Death due to: • Rx resistant disease • Transformation to Diffuse Large B Cell Lymphoma • Bad prognostic factor, less curable • Effects of Rx

  29. Management • Treat the symptoms, not the numbers • Leave the asymptomatic pt alone • Watchful waiting & repeat biopsies • ChemoRx + RadioRx • rCHOP • r = Rituximab • C = Cyclophosphamide • H = Hydroxydaunorubicin (Doxorubicin) • O = Oncovin (Vincristine) • P = Prednisolone

  30. What is Rituximab? • Trade name: Rituxan or Mabthera • Chimeric monoclonal antibody against CD20, found primarily on the surface of B cells • CD20 widely expressed on B cells, but is absent on terminally differentiated plasma cells • Basically, it induces apoptosis of CD20+ cells

  31. Diffuse Large B Cell Lymphoma • Commonest NHL (50% adults) & invariably fatal w/o Rx • Treat to cure, expectant management inappropriate • >50% of young pts are cured • Contra: Co-morbidities & pt’s will • HOPC • Rapidly progressive lymphadenopathy, symptomatic • Infiltration of extranodal organs • Morphology • Nuclei of neoplastic B cells are huge (x3-4 of resting lymphocytes)

  32. Diffuse Large B Cell Lymphoma

  33. Management • Rx decision based on staging • Cyclical chemoRx + radioRx • rCHOP • Bad prognostic factors • Disease progression during Rx • Failure to achieve complete remission during initial Rx

  34. Burkitt’s Lymphoma • Uncommon in the Western world • Endemic to Africa • Close assoc w EBV • Rapidly fatal w/o Rx • HOPC lymphadenopathy, abdo mass, BM & CNS infiltration (leukaemia & meningitis) • Morphology • Starry sky pattern • Assoc w translocations involving MYC gene

  35. Burkitt’s Lymphoma

  36. Management • Supportive Rx • Hydration • Prevent Tumour Lysis Syndrome! • Cyclical chemoRx + radioRx • rEPOCH • r = Rituximab • E = Etoposide • P = Prednisolone • O = Oncovin (Vincristine) • C = Cyclophosphamide • H = Hydroxydaunorubicin (Doxorubicin) • ?African setting

  37. Peripheral T Cell Lymphoma (Unspecified) • Most common type of adult T-cell lymphoma • Most common subtype of peripheral T Cell lymphoma • Often disseminated & aggressive

  38. Management • Treated similarly to B Cell lymphomas • Cyclical chemoRx + RadioRx • Refer to haematologist • In general, overall Rx strategies are similar but success of complete remission is ↓ than B Cell lymphomas

  39. Tumour Lysis Syndrome • Iatrogenic cause, Rx causes massive breakdown of tumour cells • Intracellular contents released into circulation • Metabolic derangement • Uric acid, potassium & phosphate • Complications • Can precipitate Acute Renal Failure • Mgmt • Identify high-risk pts • Allopurinol given prophylactically • Close monitor of electrolytes • Baseline ECGs & follow-ups

  40. Case Study #1 • 62 y/o woman referred for Ix of neck mass • HOPC • Enlarging L neck lump over last 6/52, no pain • Fatigue, unwell & LOW 3kg • O/E • Afebrile, no pallor/jaundice & peripheral oedema • BP 170/105 & prominent but undisplaced apex beat • L supraclavicular LN (firm, non-tender) • Abdo NAD, soft, tender • X2 enlarged LN in L groin • Urinalysis +1 protein • Other Info • Hx of HTN (felodipine 5mg bd) • Non-smoker & social drinker

  41. Question time • What further info should be elicited from Hx/Ex? • DDx? • Ix? • Staging the pt? • Mgmt & considerations?

  42. Interesting Fact of the Day: Breast implantation & Lymphoma • Epidemiological studies indicate link b/w silicone breast implants & anaplastic large cell lymphoma (ALCL) • ALCL devt in at least 60 women • 2/3 occurred in silicone implants • 1/3 occurred in saline implants • Considered to be related because ALCL occurred uniformly in breast tissue • FDA labelled silicone as inert, Grade 2 • Later relabelled to Grade 3 (somewhat dangerous)

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