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The Circulatory System

The Circulatory System. Cardiovascular and Lymphatic systems combined Incorporates lymphomas and leukaemias Both systems and diseases involve blood production sites Bone marrow, spleen, lymph nodes etc. BLOOD. 45% formed elements Erythrocytes Leucocytes Thrombocytes . 55% Plasma Water

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The Circulatory System

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  1. The Circulatory System • Cardiovascular and Lymphatic systems combined • Incorporates lymphomas and leukaemias • Both systems and diseases involve blood production sites • Bone marrow, spleen, lymph nodes etc ©O'Halloran Consultancy Limited

  2. ©O'Halloran Consultancy Limited

  3. BLOOD • 45% formed elements • Erythrocytes • Leucocytes • Thrombocytes • 55% Plasma • Water • Proteins • Electrolytes • Gases • Hormones • Etc ©O'Halloran Consultancy Limited

  4. Normal Blood Count • RBC • WBC • PLATELETS • 4.5-5.5 106/mm3 • 5-10 103/mm3 • 250-400 103/mm3 ©O'Halloran Consultancy Limited

  5. Diagrammatic Representation of acute and chronic leukaemia pathways ©O'Halloran Consultancy Limited

  6. Blood Cells • Erythrocytes • Carry oxygen to cells of body and CO2 away from cells • Thrombocytes (platelets) • Clotting factor ©O'Halloran Consultancy Limited

  7. White Blood Cells ©O'Halloran Consultancy Limited

  8. Hematopoietic Diseases • Characterised by an overproduction of one (sometimes more than one) type of blood cell (usually WBC) ©O'Halloran Consultancy Limited

  9. HaematopoieticDiseases • Myeloid Disease • Myelodysplastic disease • Myeloproliferative disease (Chronic) • Acute Myeloid Leukaemia (AML) • Lymphoid disease • Lymphocytic leukaemia • Lymphomas • B-cell • Myeloma (plasma cell) • T-cell ©O'Halloran Consultancy Limited

  10. Diagrammatic Representation of acute and chronic leukaemia pathways ©O'Halloran Consultancy Limited

  11. LeukaemiaIncidence • ~3% of all cancers in UK ©O'Halloran Consultancy Limited Source: ONS

  12. AETIOLOGY • ACQUIRED • Radiation • viral • chemical • cytotoxic drugs • GENETIC • Down syndrome • Siblings, twins • PH +ve (CML, AML) ©O'Halloran Consultancy Limited

  13. PATHOLOGY • ACUTE • Immature blood cells • Usually WBC • causes crowding out of normal production of RBCs and platelets • CHRONIC • mature leucocytes - but live longer than normal • enlargement of organs ©O'Halloran Consultancy Limited

  14. Typical blood picture Normal • Hb 10-14 g/dl • WBC4-10 109/l • Platelets 250 109/l Leukaemic (approx) 7-9 g/dl 10-200 109/l 30 109/l ©O'Halloran Consultancy Limited

  15. LEUKAEMIASIGNS AND SYMPTOMS • ANAEMIA • THROMBOCYTOPENIA • NEUTROPENIA • other • Tired, listless, pale • Epistaxis, haematemesis, haematuria, melaena, bruising, petechiae • Fever, infection, ulceration, • bone pain, lymphadenopathy, hepatomegaly, splenomegaly ©O'Halloran Consultancy Limited

  16. Myeloid Disease • Myeloid neoplasms consist of diseases that are commonly considered together because of their origin from the same initial differentiation pathway ©O'Halloran Consultancy Limited

  17. Myeloid Disease • These diseases manifest themselves in over- production of any one of the resultant blood cells (red, platelets, monocytes, basophils, eosinophils or neutrophils) at the expense of other blood cells. • Myelodysplastic disease • Myeloproliferative disease • Acute Myeloid Leukaemia (AML) ©O'Halloran Consultancy Limited

  18. Myelodysplastic syndromes (MDS) • Sometimes precedes true leukaemias • Group of diseases in which the production of blood cells is severely disrupted • Myelodysplasia suggests a problem with the bone marrow • Usually get over production of more than one kind--and sometimes all kinds--of blood cells ©O'Halloran Consultancy Limited

  19. Myelodysplastic Syndrome (MDS) • Refractory Anaemia • Start with abnormal red blood cells • Gradually get increased mutation of white blood cells and platelets ©O'Halloran Consultancy Limited

  20. Myelodysplastic syndromes (MDS) • Poor quality blood cells – often die before getting to circulation • Leads to panocytopenia – decrease in all types of blood cells, with associated symptoms • No real cure, treatment supportive rather than curative. May attempt BMT in highly selected patients (younger) • Often termed – pre-leukaemia – may progress to Myeloproliferative disorders ©O'Halloran Consultancy Limited

  21. Myeloproliferative Disorders • Chronic myeloid leukaemia (Ph+) • Chronic neutrophilic leukaemia • Chronic eosinophilic leukaemia • Chronic idiopathic myelofibrosis • Polycythemia vera • Essential thrombocytothemia ©O'Halloran Consultancy Limited

  22. Myeloproliferative Disorders • Over production of mature(ish) cells from the myeloid series • Typical symptoms include, enlarged spleen, fever, infection • May progress to become acute myelogenous leukaemia ©O'Halloran Consultancy Limited

  23. CHRONIC MYELOID LEUKAEMIA • 50 -60 Yrs • related to the Philadelphia Chromosome 95% of cases • Onset slow, progressive tiredness • gross splenomegaly • skin infiltration - rare • WBC 100 - 300 109/ l • ~30% myelocytes • platelets normal or raised ©O'Halloran Consultancy Limited

  24. PHASES OF CML • CHRONIC PHASE – few blast cells (<10%) • Palliative treatment or no treatment if asymptomatic • Chemo. if symptoms (hydroxycarbamide (hydroxyurea)) • Interferon, Glivec (Imatinib) or Stem cell transplants • ACCELERATED PHASE • Results of treatment v.v.poor for myeloblastic type • lymphoid type • chemo • cranial rt with intrathecal MTX • BLASTIC PHASE • Advanced stages of disease. • Very difficult to cure. • Patient usually dies within weeks due to infection, bleeding or organ failure ©O'Halloran Consultancy Limited

  25. GLIVEC (Imatinib) • Blocks the production of proteins which stimulate WBC production • 98% in Chronic phase - complete or good response • 20% in accelerated phase will regress to the chronic phase • 2 out of 3 will have disease kept under control ©O'Halloran Consultancy Limited

  26. GLIVEC (Imatinib) • Does have some side effects which some people may not be able to tolerate and therefore stop treatment • Nausea • Water retention • Puffy eyes • Vomiting • Rash • Headaches ©O'Halloran Consultancy Limited

  27. Dasatinib • May be used if developed resistance or suffering from side effect to Glivec • Not yet approved by NICE ©O'Halloran Consultancy Limited

  28. ACUTE LEUKAEMIA AML • 80% of all adult cases, mainly middle aged and elderly • associated risk with radiation exposure, workers who use benzene, people treated for ALL using alkylating agents. Smoking (?double the risk) • Can be a progression from myeloproliferative disorders ©O'Halloran Consultancy Limited

  29. ACUTE LEUKAEMIA AML • Pathology • diagnosed by bone marrow aspirate with >14% blast cells • various categories defined by the World Health Organisation • Categories based on the type of myeloid cell proliferating (see handout) ©O'Halloran Consultancy Limited

  30. Diagrammatic Representation of acute and chronic leukaemia pathways ©O'Halloran Consultancy Limited

  31. Acute Myelogenous Leukaemia • Over production of one type of myeloid immature (blast) cell which do not function as normal • Causes crowding out of red cells (anaemia), platelets (thrombocytopenia), and normal white cells (neutropenia) ©O'Halloran Consultancy Limited

  32. Management • Chemo. mainstay of treatment • Daunorubicin • Cytarabine • Bone Marrow Transplant BMT with TBI ©O'Halloran Consultancy Limited

  33. Lymphoid disease • Two main groups • B cell neoplasms • T cell neoplasms • Of these three subgroups can be identified by their individual characteristics ©O'Halloran Consultancy Limited

  34. Lymphoid disease • Lymphocytic leukemia; a lymphoid neoplasm with bone marrow involvement, accompanied by tumour cells in the peripheral blood. Lymphocytic leukaemias may suppress haematopoiesis and may also spread and infiltrate into and enlarge the spleen and liver. • Lymphoma; a proliferating discrete mass that mainly affects the lymphatic system of the body. The subtypes are Hodgkin Lymphoma (HL) and non-Hodgkin lymphoma (NHL).   • Plasma cell neoplasms; mature B-cells that are made in the marrow attack bone or soft tissues; also called myeloma. ©O'Halloran Consultancy Limited

  35. Lymphocytic Leukaemia • Neoplasms manifesting in the bone marrow • Precursor (Acute) or Mature (chronic) • B or T origin • At different stages of the differentiation pathway ©O'Halloran Consultancy Limited

  36. ACUTE LYMPHOBLASTIC LEUKAEMIA • 3/4 OF ALL CHILDHOOD LEUKAEMIAS • normally presents between 2-8 years of age • PEAK AGE - 4YRS • B:G 2:1 • ? IONISING RADIATION • ? GENETIC (20-30 fold increased incidence in those with Down Syndrome • ? IDENTICAL TWIN WITH LEUKAEMIA ©O'Halloran Consultancy Limited

  37. CLINICAL PRESENTATION • Malaise • Anaemia • Pallor • Fever • Purpura • Lymphadenopathy • Splenomegaly • Hepatomegaly • Bone, joint pain • Thrombocytopenia ©O'Halloran Consultancy Limited

  38. INVESTIGATION • Blood test • Bone marrow biopsy • Chest radiograph • Lumbar puncture • CT scan ©O'Halloran Consultancy Limited

  39. ACUTE LYMPHOBLASTIC LEUKAEMIA • Pathology • B or T cell lineage • B cell leukaemia • Precursor B • Earlier in lineage tends to be younger patient and better prognosis • T cell leukaemia • Precursor T • Male and older • Often present with enlarged thymus and lymphadenopathy ©O'Halloran Consultancy Limited

  40. MANAGEMENT • Four main phases to treatment (2-3 years to complete) • 1ST PHASE • INDUCE REMISSION WITH SUPPORTIVE THERAPY • PREDNISONE, VINCRISTINE,L-ASPARGINASE • 2ND PHASE • CNS TREATMENT: cranial irradiation and intrathecal methotrexate • 3RD PHASE • Consolidation/intensification • doxorubicin, crisantaspase, methotrexate and cytarabine • 4th PHASE • Maintenance • Oral methotrexate for 2yrs, or oral 6-mercaptopurine • Chance of relapse in testes for boys, confirm at biopsy and remove affected testis (or irradiate) ©O'Halloran Consultancy Limited

  41. CHRONIC LYMPHOCYTIC LEUKAEMIA • ? Leukaemia or low grade non-hodgkin lymphoma • M:F 2:1 • 40-50 yrs • slow progression to disease • 25% present incidentally • tiredness • enlarged lymph nodes • enlarged spleen • raised WBC usually small lymphocytes ©O'Halloran Consultancy Limited

  42. CHRONIC LYMPHOCYTIC LEUKAEMIA (CLL) • The WHO classification recognises that here are many different types of CLL and referred to as MATUREneoplasms • And that it may occur principally in the bone marrow (lymphocytic leukaemia) or the lymphatic system (lymphoma cell leukaemia) • Cross over here between leukaemia and non-hodgkins lymphoma ©O'Halloran Consultancy Limited

  43. TREATMENT • Only indicated if patient has symptoms or if critical organ involved • Chemotherapy • Local RT to bulky tumor • 50% survive 5yrs • Less than 1yr if disease is advanced ©O'Halloran Consultancy Limited

  44. PROGNOSIS5 yr survival rates Source Cancer Research UK ©O'Halloran Consultancy Limited

  45. ©O'Halloran Consultancy Limited

  46. LYMPHOMAS • A group of diseases originating in the Lymphatic system, • May also affect diffuse lymphatic tissue around the body • spleen, liver, intestines, thymus and tonsils ©O'Halloran Consultancy Limited

  47. LYMPHOMAS • HODGKIN Lymphoma • NON-HODGKIN Lymphoma ©O'Halloran Consultancy Limited

  48. HODGKIN LYMPHOMAEPIDEMIOLOGY • PROGNOSIS IMPROVING OVER LAST 25 YRS • better understanding of RT and Chemo. • better localisation and staging methods • better understanding of patterns of spread ©O'Halloran Consultancy Limited Reproduced with permission from the Office of National Statistics,

  49. HODGKIN LYMPHOMAEPIDEMIOLOGY • Bipolar age of incidence inc. • M:F 3:2 (West) • more common in higher social class ©O'Halloran Consultancy Limited

  50. HODGKIN LYMPHOMAAetiology • Unclear as to what causes HL • Some ideas include • genetic susceptibility • siblings at greater risk if member of family has HL • Twins are at 100x risk • infection • EBV • reduced immunity • HIV • transplants ©O'Halloran Consultancy Limited

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