7.2. Haematological Malignancies - Lymphoid Malignancies Flashcards

1
Q

What does the Multipotential Haematopoietic Stem Cell Divide into?

A
  1. Common Myeloid Progenitor

2. Common Lymphoid Progenitor

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2
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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3
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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4
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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5
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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6
Q

What are the Types of Acute Leukaemias?

A
  1. Acute Lymphoblastic Leukaemia (ALL)

2. Acute Myeloid Leukaemia (AML)

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7
Q

What are the Types of Chronic Leukaemias?

A
  1. Chronic Lymphocytic Leukaemia

2. Chronic Myeloid Leukaemia

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8
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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9
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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10
Q

What is the Difference Between:

  1. Hodgkin Lymphoma?
  2. Non-Hodgkin Lymphoma?
A
  1. Hodgkin Lymphoma is a Specific Disease

2. Non-Hodgkin Lymphoma is everything else - 50 subtypes

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11
Q

What are the Major Lymphoproliferative Disorders?

A
  1. Acute Lymphoblastic Leukaemia - Rare
  2. Chronic Lymphocytic Leukaemia
  3. Hodgkin Lymphoma
  4. High-Grade Non-Hodgkin Lymphoma - 1/3
  5. Low-Grade Non-Hodgkin Lymphoma - 1/3
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12
Q

What is Acute Lymphoblastic Leukaemia?

A

Neoblastic Disorder of Lymphocytes

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13
Q

What are the Features of Acute Lymphoblastic Leukaemia?

A
  1. 75% cases in children < 6 years old
  2. 75-90% are B-Cell Lineage
  3. Presents with a 2-3 week history of Bone Marrow Failure / Bone Pain / Joint Pain
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14
Q

What are the Laboratory Findings for Acute Lymphoblastic Leukaemia?

A
  1. Low Haemoglobin
  2. High White Cell Count
  3. Low Platelets
  4. > 20% B-Lymphocytes present in Bone Marrow
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15
Q

What is the Treatment of Acute Lymphoblastic Leukaemia?

A
  1. Chemotherapy
  2. Consolidation Therapy
  3. CNS Directed Treatment
    Note - Maintenance Treatment for 18 months
  4. Stem Cell Transplantation - if High Risk
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16
Q

What are the Poor Risk Factors for Acute Lymphoblastic Leukaemia?

A
  1. Increasing Age
  2. Increased White Cell Count
  3. Immunophenotype (more primitive forms)
  4. Cytogenetics / Molecular Genetics
  5. Slow / Poor Response to Treatment
17
Q

What are the outcomes for:

  1. Adults with Acute Lymphoblastic Leukaemia?
  2. Children with Acute Lymphoblastic Leukaemia?
A
  1. a) Complete Remission Rate = 78-91%
  2. b) Leukaemia Free Survival at 5 years = 30-35%
  3. a) 5 year Overall Survival = 90%
  4. b) Poor Risk Patients 5 year = 45%
18
Q

What is the Clinical Presentation of Chronic Lymphocytic Leukaemia?

A
  1. Asymptomatic
  2. Bone Marrow Failure - Anaemia / Thrombocytopenia
  3. Lymphadenopathy
  4. Hepato/Splenomegaly
  5. Fever / Sweats / Weight Loss
  6. Infections / Immune Paresis
  7. Haemolytic Anaemia
19
Q

What are the Laboratory Findings for Chronic Lymphocytic Leukaemia?

A
  1. Blood > 5 x 10^9 / L Lymphocytes
  2. Bone Marrow > 30% Lymphocytes
  3. Characteristic Immunophenotyping - B-Cell Markers
20
Q

What is the Staging Method used for Chronic Lymphocytic Leukaemia?

A

Binet:
Stage A - <3 Lymph Node Areas - Same Median Survival
Stage B - 3+ Lymph Node Areas - 8 year Median Survival
Stage C - Stage B + Anaemia / Thrombocytopenia - 6 year Median Survival

21
Q

What are the Indications for Treatment of Chronic Lymphocytic Leukaemia?

A
  1. Progressive Bone Marrow Failure
  2. Massive Lymphadenopathy
  3. Progressive Splenomegaly
  4. Lymphocyte Doubling Time < 6 Months
  5. > 50% Lymphocyte Increase over 2 Months
  6. Systemic Symptoms
  7. Autoimmune Cytopenias
22
Q

What is the Treatment of Chronic Lymphocytic Leukaemia?

A
  1. Watch and Wait
  2. Cytotoxic Chemotherapy - Fluarabine / Bendamustine
  3. Monoclonal Antibodies - Rituximab / Obinutuzamab
  4. Novel Agents - Tyrosine Kinase Inhibitor
  5. Bone Marrow Transplant
23
Q

What are the Poor Prognostic Markers of Chronic Lymphocytic Leukaemia?

A
  1. Advanced Disease
  2. Atypical Lymphocyte Morphology
  3. Rapid Lymphocyte Doubling Time
  4. CD38+ Expression
  5. Loss / Mutation p53
24
Q

What is the Clinical Presentation of a Lymphoma?

A
  1. Lymphadenopathy / Hepatosplenomegaly
  2. Extranodal Disease
  3. “B Symptoms”
  4. Bone Marrow Involvement
25
Q

How is a Lymphoma Staged?

A
  1. Lymph Node Biopsy
  2. CT Scan
  3. Bone Marrow Aspirate and Trephine
26
Q

What is a Non-Hodgkin Lymphoma Classified According to?

A
  1. Lineage - B-Cell / T-Cell
  2. Grade of Disease - High-Grade / Low-Grade
  3. Histological Features
27
Q

What are the Features of a Low Grade Non-Hodgkin Lymphoma?

A
  1. Indolent - Often Asymptomatic
  2. Responds to Chemotherapy but Incurable
  3. Median Survival Rate Varies by Subtype
28
Q

What are the Features of a High Grade Non-Hodgkin Lymphoma?

A
  1. Aggressive - Fast Growing
  2. Require Combination Chemotherapy
  3. Can be Cured, but Varies Widely
29
Q

What are the Specific Disease Entities of Non-Hodgkin Lymphoma?

A
  1. Diffuse Large B-Cell Lymphoma - High Grade Lymphoma

2. Follicular Lymphoma - Low Grade Lymphoma

30
Q

What is the Treatment of Non-Hodgkin Lymphoma?

A

Combination Chemotherapy:

  1. Anti-CD20 Monoclonal Antibody
  2. Chemo
31
Q

What is Hodgkin Lymphoma associated with?

A
  1. Epstein Barr Virus
  2. Familial Clustering
  3. Geographical Clustering
32
Q

What is the Treatment of Hodgkin Lymphoma?

A
  1. Combination Chemotherapy
  2. +/- Radiotherapy
  3. Use of PET Scan to assess Response to Treatment