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Pathology Course Haematology 2

Pathology Course Haematology 2. Kindly sponsored by:. What will be discussed. Tuesday: Anaemia, coagulation, thrombosis Thursday: Haematological malignancies Paraproteinaemias Myelodysplastic / myeloproliferative disorders. General tips for written exams. Don’t focus too much on

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Pathology Course Haematology 2

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  1. Pathology Course Haematology 2 Kindly sponsored by:

  2. What will be discussed • Tuesday: Anaemia, coagulation, thrombosis • Thursday: • Haematological malignancies • Paraproteinaemias • Myelodysplastic / myeloproliferative disorders

  3. General tips for written exams • Don’t focus too much on • Eponymous syndromes • Nitty gritty facts e.g. chromosomal translocations • Do WebCT questions • Some questions will be hard / strange • Understanding is key

  4. Acute Leukaemia

  5. Acute Leukaemia • Neoplastic process of bone marrow and blood • ‘Acute’ therefore rapidly progressive and fatal • Immature blasts > 20% BM cells • Signs/symptoms: • BM failure: anaemia, thrombocytopenia, neutropenia • Organ infiltration: hepato/spleno-megaly, lymphadenopathy, bone pain, gum hypertrophy

  6. Aetiology • Ionising radiation – radiotherapy • Cytotoxic drugs – chemotherapy • Benzene • Pre-leukaemic disorders e.g. MDS/MPD • Down’s • Significnatly increased risk of AML/ALL • Also, Down’s neonates often develop transient abnormal myelopoeisis • Resembles AML • Resolves spontaneously after a few weeks

  7. Acute Lymphoblastic Leukaemia • Childhood (ALL) • Symptoms as previously mentioned • Lymphadenopathy +++ • CNS involvement +++ • Testicular enlargment • Thymic enlargement • High WCC (accumulation of lymphoblasts) • Ph+ve t(9;22) – poor prognosis

  8. ALL: Treatment • Chemotherapy: (memory aid: ICan Cure Mankind) • Remission Induction • Consolidation • CNS treatment • Maintenance • Supportive • Blood products, fluids, electrolyes

  9. Acute Myeloid Leukaemia • Adulthood (risk increases with age) and under 2’s • Signs/symptoms as before • Lymphadenopathy less common • Subtypes • M3: Acute promyelocyticleukaemia DIC • M5: Skin/gum infiltration + hypokalaemia

  10. AML: Lab High WCC Auer rods and granules Myeloperoxidase and Sudan black +ve

  11. AML: Treatment • Chemotherapy • Similar principles to ALL but… • No CNS prophylaxis or maintenance needed • ATRA for M3 • Consider allo-SCT in young

  12. Chronic Myeloid Leukaemia

  13. Chronic Myeloid Leukaemia A myeloproliferative disease Often diagnosed on routine bloods Now very successfully treated in the majority

  14. CML: Presentation 40-60 years peak (middle-aged) May be asymptomatic Anaemia Weight loss, low grade fever, night sweats Massive splenomegaly (down to RIF) Gout (raised urate – hypermetabolism)

  15. CML: Lab • Huge WCC > 100 (neutrophilia) • Hypercellular BM with spectrum of mature and immature cells in the blood • Malignant cells proliferate excessively but retain ability to differentiate into mature cells • Ph+ve in 80%; t(9;22) • PCR for BCR-ABL • Monitor disease and therapeutic response

  16. CML: Phases & Treatment Chronic Phase • < 5% blasts in BM/blood • WBC slowly increases • Over 3-4 years • Rx = Imatinib • BCR-ABL tyrosine kinase inhibitor

  17. CML: Phases & Treatment Accelerated phase > 10% blasts in BM/blood Increasing manifestations e.g. splenomegaly Up to a year Less responsive to therapy

  18. CML: Phases & Treatment Blast phase > 20% blasts in BM/blood Resembles an acute leukaemia Course = months Treatment similar to AML

  19. Chronic Lymphocytic Leukaemia

  20. Chronic Lymphocytic Leukaemia • A lymphoproliferative disease • Others include ALL, MM and Waldenstrom’s • Also often an incidental finding on routine bloods • Disease of the elderly

  21. CLL & SLL Chronic lymphocytic leukaemia and small lymphocytic lymphoma Same underlying disease, different presentation CLL is primarily located in BM SLL is primarily located in lymph nodes

  22. CLL: Presentation • > 40 years, elderly • May be asymptomatic • Symmetrical painless lymphadenopathy • Anaemia, thrombocytopenia, neutropenia • Wt loss, low grade fever, night sweats • Hepato/Spleno-megaly (less prominent) • Ass w/ autoimmunity • AIHA (Coomb’s +ve) + ITP = Evan’s syndrome

  23. CLL: Lab • High WCC with lymphocytosis > 5 (high % lymphocytes) • Low serum Ig • ‘Smear cells’ • Prognostic factors • LDH raised = bad • HypermutatedIg gene = good

  24. CLL: Stages & Treatment • Binet Staging A, B & C • Stage A • < 3 groups of enlarged LN • No treatment required • Stage B & C • > 3 groups of LN, patient becomes more symptomatic • Rx: 1st line is chlorambucil

  25. CML vs CLL CML CLL Middle-aged Massive splenomegaly Neutrophilia Ph+ve, BCR-ABL fusion gene Imatinib Rx Older AID Lymphocytosis Smear cells Chlorambucil Rx

  26. Lymphoma

  27. Lymphoma • Neoplastictumour of lymphoid tissue • Lymph nodes • Spleen, MALT • Rarely anywhere – skin, testes, breast • Hodgkin’s – 20% • Non- Hodgkin’s – 80%

  28. Hodgkin’s lymphoma M>F Bimodal incidence EBV associated Spreads to adjacent lymph nodes – often involves single LN group Assymetrical painless lymphadenopathy Constitutional symptoms (Pel-Ebstein fever in a minority) Pain after alcohol in affected nodes

  29. Presentation CXR Mediastinal mass: Cough & resp infections SVC syndrome Dysphagia Normal nk

  30. Hodgkin’s: lab • CD15 & CD30 • Reed-Sternberg cell: binucleate / multinucleate cell on background of lymphocytes • Subtypes • Nodular sclerosing – most common

  31. Staging Stage I: 1 lymph node region Stage II: 2+ LN region; same side of diaphragm Stage III: 2+ LN regions; opposite sides of diaphragm Stage IV: Extranodal sites, e.g. liver/BM A: No constitutional symptoms B: Constitutional symptoms

  32. Treatment • Combination chemotherapy • ABVD – adriamycin, bleomycin, vinblastine, dacarbazine • 2-4 cycles in I/II, 6-8 cycles in III/IV • Radiotherapy • Sometimes used alongside chemo against bulky areas • Intensive chemo + autologous SCT • Relapsed patients • ESR used for progress and monitoring

  33. Non-Hodgkin’s Lymphoma • All lymphomas other than Hodgkin’s • Approximately 40 different subtypes • Mature/Immature • Histology • High grade • Very aggressive – Burkitt’s • Aggressive – Diffuse Large B-Cell, Mantle Cell • Low grade • Indolent – Follicular, Marginal Zone, Small Lymphocytic • Lineage • B-Cell: All of the above • T-Cell

  34. Non-Hodgkin’s Lymphoma • Presentation varies significantly from subtype to subtype • Painless lymphadenopathy • Often involves multiple sites • Constitutional symptoms • No pain after alcohol • Staging as per Hodgkin’s

  35. Burkitt’s Lymphoma • Very aggressive • Three variants • Endemic – most common malignancy in equatorial Africa; EBV associated; jaw involvement and abdominal masses • Sporadic – found outside of Africa, EBV associated, less commonly affects jaw • Immunodeficiency – HIV/Post-Tx patients; not EBV associated • Histology • ‘Starry-sky’ appearance • t(8;14) translocation; c-myconcogeneoverexpression • Treatment – chemotherapy (rituximab)

  36. Diffuse Large B-Cell Lymphoma (DLBCL) • Middle-aged and elderly • Aggressive • Richter’s transformation: leukaemias DLBCL • Histology • Sheets of large lymphoid cells • Treatment • Rituximab-CHOP

  37. Mantle Cell Lymphoma • Middle-aged • Aggressive • Disseminated at presentation • Median survival 3-5 years • Histology • ‘Angular nuclei’ • t(11;14) translocation • Dysregulation of cyclin D1 • Tx similar to DLBCL

  38. Follicular Lymphoma • Elderly • Indolent • Median life expectancy 12-15 years • Mostly incurable • Histology • ‘Follicular pattern’, ‘Nodular appearance’ • t(14;18) translocation • Treatment • Watch and wait • Rituximab-CVP

  39. MALT lymphomas • Mucosal Associated Lymphoid Tissue • Marginal Zone NHL • Middle aged • Chronic antigen stimulation • H. Pylori  gastric MALT lymphoma • Sjogren’s syndrome  parotid lymphoma • Treatment • Remove antigenic stimulus e.g. H pylori triple therapy • Chemotherapy

  40. T-Cell Lymphomas • Adult T-Cell leukaemia/lymphoma • HTLV-1 infection • Enteropathy associated T-cell lymphoma • Long standing coeliac disease • Cutaneous T-cell lymphoma • Anaplastic large cell lymphoma • Children and young adults • Aggressive • Large ‘epitheloid’ lymphocytes • Peripheral T-Cell Lymphoma • Middle aged and elderly • Aggressive • Large T cells

  41. Question Time! A. CML B. AML C. ALL D. Normal E. CLL F. Mantle Cell Lymphoma G. Hodgkin’s H. Follicular Lymphoma A routine medical of a 33 year old footballer reveals: Hb 9.9, WCC 130. Blood film shows spectrum of myeloid precusors including a few blast cells. He admits to having frequent night sweats and blurred vision. Cytogenetic analysis shows Ph chromosome t(9;22)

  42. Question Time! A. CML B. AML C. ALL D. Normal E. CLL F. Mantle Cell Lymphoma G. Hodgkin’s H. Follicular Lymphoma A routine medical of a 33 year old footballer reveals: Hb 9.9, WCC 130. Blood film shows spectrum of myeloidprecusors including a few blast cells. He admits to having frequent night sweats and blurred vision. Cytogenetic analysis shows Ph chromosome t(9;22)

  43. Question Time! A. CML B. AML C. ALL D. Normal E. CLL F. Mantle Cell Lymphoma G. Hodgkin’s H. Follicular Lymphoma A 5 year old girl presents with failure to thrive, recurrent fever and bruising. Immunophenotyping reveals the presence of CD10.

  44. Question Time! A. CML B. AML C. ALL D. Normal E. CLL F. Mantle Cell Lymphoma G. Hodgkin’s H. Follicular Lymphoma A 5 year old girl presents with failure to thrive, recurrent fever and bruising. Immunophenotyping reveals the presence of CD10.

  45. Question Time! A. CML B. AML C. ALL D. Normal E. CLL F. Mantle Cell Lymphoma G. Hodgkin’s H. Follicular Lymphoma A 64 year old woman receiving long-term chemotherapy for lymphoma presents with worsening bone pain, recurrent fever and night sweats. Blood film shows blast cells and Aeur rods.

  46. Question Time! A. CML B. AML C. ALL D. Normal E. CLL F. Mantle Cell Lymphoma G. Hodgkin’s H. Follicular Lymphoma A 64 year old woman receiving long-term chemotherapy for lymphoma presents with worsening bone pain, recurrent fever and night sweats. Blood film shows blast cells and Aeur rods.

  47. Question Time! A. CML B. AML C. ALL D. Normal E. CLL F. Mantle Cell Lymphoma G. Hodgkin’s H. Follicular Lymphoma An 85 year old lady with a painless swelling in her neck. After undergoing several investigation, she is told that there is cancer present but it is very slow growing. She is advised that she will be followed up regularly but no intervention is necessary at present.

  48. Question Time! A. CML B. AML C. ALL D. Normal E. CLL F. Mantle Cell Lymphoma G. Hodgkin’s H. Follicular Lymphoma An 85 year old lady with a painless swelling in her neck. After undergoing several investigation, she is told that there is cancer present but it is very slow growing. She is advised that she will be followed up regularly but no intervention is necessary at present.

  49. Multiple Myeloma

  50. Multiple Myeloma Neoplasia of plasma cells of BM Production of monocloncalIg (IgG most common) = paraproteinaemia Middle aged – elderly Increased incidence in Afro-Caribbeans

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