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Haematology

Haematology . By Matthew Alley. History . None specific features- fever, weight loss, fatigue Anaemic symptoms- reduced exercise capacity, breathlessness, worsening angina Neutropenia symptoms- oral ulceration, oral sepsis, skin infections Compromised immunity- recurrent infections

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Haematology

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  1. Haematology By Matthew Alley

  2. History • None specific features- fever, weight loss, fatigue • Anaemic symptoms- reduced exercise capacity, breathlessness, worsening angina • Neutropenia symptoms- oral ulceration, oral sepsis, skin infections • Compromised immunity- recurrent infections • Haemostatic problems- bleeding, bruising, rashes • Anatomical symptoms- abdominal pain (from splenomegaly), headache (CNS lymphoma)

  3. Clinical Exam • General Examination- weight loss, pallor, SOB, jaundice, abnormal pigmentation, rashes • Eyes- pallor of conjunctive, fundoscopy may reveal haemorrhage • Mouth- oral ulceration, purpura, gum bleeding/ hypertrophy • Chest and abdomen- lymph nodes, hepatosplenomegaly • Bones and joints- swelling, tenderness • Neurological involvement may present with signs

  4. Definitions • Haematopoiesis • Formation of the cellular components of the blood, occurs in bone marrow • Erythrocyte • Other name for a red blood cell • Granulocytes • White blood cells characterised by their granular cytoplasm: Neutrophils, Eosinophils, Basophils • Neutrophil • Most common phagocytic granulocyte found in circulation. Helps defend against bacteria and fungi • Eosinophil • Role in allergy and defence against parasites • Basophil • Role in inflammatory response • Lymphocyte • Most abundant white blood cell. Can be split into B (antibody producing cells), helper T, killer T and NK. Released from the Marrow and continue to change in lymph nodes/ thymus in response to pathogens • Blasts • Immature cells normally found in the bone marrow • Monocyte • White blood cell that phagocytoses pathogens, longer lived than neutrophils • Megakaryocyte • Large cell found in the bone marrow. Responsible for platelet production • Paraprotein • The immunoglobulin produced in excess in conditions such a multiple myeloma

  5. Conditions • Anaemia • Microcytic • Normocytic • Macrocytic • Haemolytic • Inherited Conditions • Sickle Cell Anaemia • Thalassemia • Bleeding Disorders • Myelodysplasia • Myeloproliferative Neoplasms • Polycythaemia Rubra Vera • Thrombocythaemia • CML • Myelofibrosis • Leukaemia • AML/ALL • CML/ CLL • Lymphoma • Hodgkin's • Non-Hodgkin’s • Paraproteinaemias • Multiple Myeloma • MGUS • Waldenstroms Macroglobulinaemia • Amyloidosis • Pancytopenia and Bone Marrow Failure • Bone Marrow Transplant • Graft vs. Host Disease

  6. Myeloproliferative Neoplasms • Chronic neoplasms resulting in effective overproduction of mature cells of myeloid lineage • “Myelo”- the lineage of cells • “Proliferative”- implying increased numbers of cells • Patients have an increased risk of Myelofibrosis and AML • Hyper-cellular marrow • JAK-2 mutation common to all MPNs

  7. Polycythaemia Rubra Vera • Increased production of erythrocytes • 95% have acquired JAK-2 mutation • Symptoms: • Headache • Dizziness • TIA/ Stroke • Gangrene • MI • Splenomegaly • Normally have a ‘plethoric’ complexion • Complicated by thromboembolic events • Can transform into Myelofibrosis and less commonly AML • Treated with venesection, aspirin and chemotherapy (may increase risk of AML transformation)

  8. Essential Thrombocythaemia • Characterised by persistently elevated platelets • 50% are JAK-2 +ve • Risk of transformation into AML is very low • Best prognosis out of all the MPNs • Life expectancy is near normal • Diagnosis is often incidental, symptoms: • Headache • Chest pain • Light headedness • Increased risk of thromboembolic events • Patients can be managed with aspirin, chemotherapy increases the risk of transformation to AML

  9. Myelofibrosis • Fibrosis of the marrow secondary to hyperplasia of abnormal megakaryocytes • Can occur at any stage of the other MPNs • Presents with symptoms of bruising, anaemia and hepatosplenomegaly • In later stages patients may present with bone marrow failure • The marrow starts to secrete immature cells into circulation (extra medullary haematopoiesis) • Morbidity and mortality commonly due to AML transformation

  10. Myelofibrosis

  11. Chronic Myeloid Leukaemia • Disease of middle age • Increased circulation of mature granulocytes • Occurs as a result of BCR-ABL fusion gene (aka the Philadelphia chromosome) • Symptoms include: • Fatigue • Lethargy • Bleeding • Weight loss • Splenomegaly • May be an incidental finding • 3 phases • Chronic phase • Accelerated phase  blood counts and clinical picture increasingly refractory to therapy • Blast crisis  resembles acute leukaemia (may emerge directly from chronic phase) • Treated with tyrosine kinase inhibitors (Imatinib) • In disease refractory to Imatinib a stem cell transplant may be indicated

  12. Chronic Myeloid Leukaemia Blood Film

  13. Myelodysplastic Syndromes • Typically a disease of the elderly • Group of disorders characterised by ineffective haematopoiesis resulting in the production morphologically and functionally abnormal cells • Can lead to: • Peripheral cytopenia • Bone marrow failure • AML • Clinical presentation can range from mild anaemia to pancytopenia • Common presentation is microcytic anaemia refractory to therapy • Disease of the elderly • Those who have received cancer therapy in the past are at a higher risk

  14. Myelodysplasia

  15. Myelodysplastic Syndromes • Symptoms arise from the lack of functioning cells • Red blood cells- anaemia, lethargy, pallor, breathlessness • Granular cells- recurrent infections, mouth ulcers • Platelets- bruising, bleeding • Treatment largely depends on the performance status of the patient • Red cell transfusion • Platelet transfusion • Granular colony stimulating factor • Erythropoietin • Intensive therapy  chemotherapy +/- stem cell transplant

  16. Leukaemia

  17. Acute Myeloid Leukaemia • Bone marrow malignancy in cells of non-lymphoid lineage • More common in adults • Normally sporadic • Can arise from a background of Myelodysplasia and Myeloproliferative disorders • Signs/ symptoms • Hepatosplenomegaly • Lymphadenopathy • Gum hypertrophy • Features of marrow failure (bleeding, bruising, anaemia, petechial rash, fever) • ‘Auer Rod’ on blood film is diagnostic

  18. Auer Rod

  19. Treatment and Prognosis of AML • Treatment is with intensive chemotherapy followed by a bone marrow transplant • The specific chemotherapeutic agent is determined by the specific type of AML • Prognosis depends on specific mutations • The average AML patient has a 50% 5 year survival rate and a 50% chance of relapse

  20. Acute Lymphoblastic Leukaemia • Commonest cancer of childhood • 85% of children with this disease are cured with modern therapeutic agents • Prognosis is much worse in adults with 5 year survival being 45%, and 10% for those over 60 • Signs/ symptoms • Bone pain • Lymphadenopathy, Hepatosplenomegaly • Testicular infiltration • CNS infiltration leading to cranial nerve palsies • Features of marrow failure (bleeding, bruising, anaemia, petechial rash, fever, chest infection) • Sweats • Treated with high dose chemotherapy and bone marrow transplant depending on the specific type of leukaemia

  21. Acute Lymphoblastic Leukaemia

  22. Chronic Lymphocytic Leukaemia • Commonest type of leukaemia • Disease of middle to old age • Results from proliferation of mature B-Cells • Signs/ symptoms • Asymptomatic • Lymphadenopathy • Splenomegaly • Increased susceptibility to infections • In a small number of patients CLL can transform into lymphoma • Many patients do not require treatment • Some may be treated with immunosuppressive agents such as fludarabine

  23. Lymphoma B-Cell Hodgkin’s LYMPHOMA T-Cell B-Cell Non-Hodgkin’s NK-Cell

  24. Non-Hodgkin’s Lymphoma • All lymphomas that don’t have ‘Reed-Sternberg Cells’ • Accounts for roughly 85% of lymphomas • Diverse group of diseases • Incidence is on the rise for unknown reasons • Signs/ Symptoms • Lymphadenopathy • Fever • Night sweats • Weight loss • Diagnosis is made on biopsy • Paradoxically aggressive lymphomas are more likely to be cured than less aggressive ones. This is due to their rapid presentation and sensitivity to chemotherapy • Treated with radiotherapy/ chemotherapy • Worse prognosis than HL, 30-50% 5 year survival

  25. Hodgkin’s Lymphoma • Malignancy of lymphocytes • Rarer than NHL • Includes all lymphomas with Reed Sternberg cells (diagnostic) • Symptoms include: • Painless rubbery lymphadenopathy • Fever • Weight loss • Lethargy • Pruritus • Sweats • Diagnosed on biopsy • Treated with chemotherapy • Patients have a better prognosis than they do in NHL, 10 year survival ranges from 50-80% depending on stage

  26. Reed-Sternberg Cells

  27. Ann Arbor Staging of Lymphoma

  28. Multiple Myeloma

  29. Multiple Myeloma • Clonal Plasma cell malignancy that secretes monoclonal antibody (aka paraprotein) • Peak incidence age 60 • More commonly seen in Afro-Caribbean populations • Symptoms/ signs • Bone pain/ bony tenderness (due to osteolytic lesions) • Pathological fractures • Respiratory infection • Anaemia/ pallor/ bruising/ bleeding • Can be complicated by renal failure, amyloidosis, spinal chord compression, bone fractures and hyperviscosity

  30. Multiple Myeloma

  31. Multiple Myeloma Investigations/ Treatment • Ix • Raised creatinine, urea, calcium, ESR • Bence-Jones proteins may be seen in the urine • Radiology may reveal osteolytic lesions of the bones • Rx • Analgesia • Radiotherapy to the bony lesions • Bisphosphonates • Steroids • Chemotherapy • Plasmapheresis • Thalidomide • Bone marrow transplant (Autologous) • Median survival is 3-5 years

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