7.1. Haematological Malignancies - Introduction and Myeloid Malignancies Flashcards

1
Q

What is the Epidemiology of Haematological Malignancies?

A
  1. 10% of All Human Cancers
  2. Occur In All Age Groups - Peak incidence of Hodgkin Lymphoma = 18-35 vs Myelodysplastic Syndromes = 80’s
  3. Males more commonly affected than Females
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2
Q

What does the Multipotential Haematopoietic Stem Cell Divide into?

A
  1. Common Myeloid Progenitor

2. Common Lymphoid Progenitor

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3
Q

What does the Common Myeloid Progenitor (From the Multipotential Haematopoietic Stem Cell) divide into?

A
  1. Megakaryocyte - THROMBOCYTES
  2. ERYTHROCYTES
  3. MAST CELLS
  4. Myeloblast:
  5. a) BASOPHIL
  6. b) NEUTROPHIL
  7. c) EOSINOPHIL
  8. d) Monocyte - MACROPHAGE
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4
Q

What does the Common Lymphoid Progenitor (From the Multipotential Haematopoietic Stem Cell) divide into?

A
  1. NATURAL KILLER CELL (Large Granular Lymphocyte)
  2. Small Lymphocyte:
  3. a) T-LYMPHOCYTE
    2 b) B-LYMPHOCYTE
  4. c) PLASMA CELL
    Note - The B-Lymphocyte goes on to form the Plasma Cell
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5
Q

What is the Difference between Leukaemia and Lymphoma?

A

These are Descriptive Terms or Presentation of the Same Disease:

  1. Leukaemia - Predominantly in the Bone Marrow / Blood
  2. Lymphoma - Predominantly in the Lymph Nodes / Solid Organs
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6
Q

What are the Major Groups of Haematological Malignancies?

A
  1. Acute Leukaemias
  2. Chronic Leukaemias
  3. Malignant Lymphomas
  4. Multiple Myeloma
  5. Myelodysplastic Syndromes (MDS)
  6. Chronic Myeloproliferative Diseases (Biologically Malignant)
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7
Q

What are the Types of Acute Leukaemias?

A
  1. Acute Lymphoblastic Leukaemia (ALL)

2. Acute Myeloid Leukaemia (AML)

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8
Q

What are the Types of Chronic Leukaemias?

A
  1. Chronic Lymphocytic Leukaemia

2. Chronic Myeloid Leukaemia

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9
Q

Where do the Major Groups of Haematological Malignancies occur?

A

Haemopoietic Stem Cell:

  1. Lymphoid Progenitor Cell - Acute Lymphblastic Leukaemia:
  2. a) B-Lymphocytes - Chronic Lymphocytic Leukaemia
  3. a) i) Geminal Centre - Lymphomas
  4. a) i) 1) Plasma Cells - Mulitple Myeloma
  5. b) T-Lymphocytes - Lymphomas
  6. Myeloid Progenitor Cell - Acute Myeloid Leukaemia
  7. a) Myeloproliferative Disorders
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10
Q

What is the Difference between Acute and Chronic Leukaemia?

A

Acute vs Chronic:

  1. No Differentiation vs Retained Ability to Differentiate
  2. Bone Marrow Failure vs Proliferation without Bone Marrow Failure
  3. Rapidly Fatal vs Survival of a Few Years if Untreated
  4. Curable vs Uncurable without BMT
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11
Q

What are the indications of Lymphadenopathy being:

  1. Localised and Painful?
  2. Localised and Painless?
  3. Generalised and Painful / Tender?
  4. Generalised and Painless?
A
  1. Bacterial Infection in Draining Site
  2. Metastatic Carcinoma / Lymphoma / Rare Infections / Reactive (no cause identified)
  3. Viral Infections - EBV / CMV / Hepatitis / HIV
  4. Lymphoma / Leukaemia / Connective Tissue Disease / Drugs / Reactive (no cause identified)
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12
Q

What are the Clinical Features of a Multiple Myeloma (Plasma Cell Malignancy)?

A
  1. Bone Pain and Lytic Lesions
  2. Anaemia
  3. Recurrent Infetcion
  4. Renal Failure
  5. Amyloidosis
  6. Bleeding Tendency
  7. Hyperviscocity Syndrome
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13
Q

What are the Clinical Features of Acute Myeloid Leukaemia?

A
  1. Bone Marrow Failure
  2. Anaemia
  3. Thrombocytopenic Bleeding - Purpura / Mucosal Membrane
  4. Infection because of Neutropenia - Bacterial / Fungal
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14
Q

What are the Essential Investigations for Acute Myeloid Leukaemia?

A
  1. Blood Count / Blood Film
  2. Bone Marrow Aspirate / Trephine
  3. Cytogenetics of Leukaemic Blasts
  4. Immunophenotyping of Leukaemic Blasts
  5. CSF Examination, if Symptomatic
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15
Q

What is the Treatment of Acute Myeloid Leukaemia?

A
  1. Supportive Care
  2. Anti-Leukaemic Chemotherapy
  3. Stem Cell Transplantation - Allogenic
  4. All-Trans Retinoic Acid - in Acute Promyelocytic Leukemia
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16
Q

What are the Clinical Features of Chronic Myeloid Leukaemia?

A
  1. Anaemia
  2. Splenomegaly - often Massive
  3. Weight Loss
  4. Hyperleukostasis - Fundal Haemorrhage / Venous Congestion / Altered Consciousness / Respiratory Failure
  5. Gout
17
Q

What are the Laboratory Features of Chronic Myeloid Leukaemia?

A
  1. High White Cell Count
  2. High Platelet Count
  3. Anaemia
  4. Blood Film shows all stages of White Cell Differentiation with Increased Basophils
  5. Bone Marrow is Hypercellular
  6. Bone Marrow and Blood Cells contain the Philadelphia Chromosome
18
Q

What is the Treatment of Chronic Myeloid Leukaemia?

A
  1. Tyrosine Kinase Inhibitors - Imatinib / Dasatinib / Nilotinib
  2. Allogenic Transplantation - if above fails
19
Q

What are the Features of Myelodysplastic Syndromes?

A
  1. Acquired Clonal Disorders of the Bone Marrow
  2. Commonly seen in old age
  3. Fatal as a Result of Pregression to Bone Marrow Failure / AML
20
Q

What is the Presentation of Myelodysplastic Syndromes?

A
  1. Macrocytic Anaemia
  2. Pancytopenia
  3. Pre-Leukaemic
21
Q

What is the Treatment of Myelodysplastic Syndromes?

A
  1. Supportive

2. Stem Cell Transplantation - for the young patients

22
Q

What are the Common Myeloproliferative Diseases?

A
  1. Polycythaemia Vera
  2. Essential Thrombocythaemia
  3. Idiopathic Myelofibrosis
23
Q

What are the Clinical Features of Polycythaemia Vera?

A
  1. Headaches
  2. Itch
  3. Vascular Occlusion
  4. Thrombosis
  5. TIA / Stroke
  6. Splenomegaly
24
Q

What are the Laboratory Features of Polycythaemia Vera?

A
  1. Raised Haemoglobin Concentration / Haematocrit
  2. Raised WCC / Platelet Count
  3. Raised Uric Acid
  4. True increase in Erythrocyte Mass
25
Q

What is the Treatment of Polycythaemia Vera?

A
  1. Venesection - to keep the Haematocrit Low
  2. Aspirin
  3. Hydroxcarbamide
26
Q

What is the Natural History of Polycythaemia Vera?

A
  1. Stroke / Thrombus if poorly controlled
  2. Bone Marrow Failure - from the Secondary Myelofibrosis
  3. Transformation to AML
27
Q

What are the Features of Essential Thrombocythaemia?

A
  1. Myeloproliferative Disease with the Predominant Feature of Raised Platelet Count
  2. Arterial / Venous Thromboses
  3. Digital Ischaemia
  4. Gout
  5. Mild Splenomegaly
    Note - Can Progress to Myelofibrosis / AML
28
Q

What is the Treaatment of Essential Thrombocythaemia?

A
  1. Aspirin

2. Hydroyurea