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
How is a Lymphoma Staged?
1. Lymph Node Biopsy 2. CT Scan 3. Bone Marrow Aspirate and Trephine
26
What is a Non-Hodgkin Lymphoma Classified According to?
1. Lineage - B-Cell / T-Cell 2. Grade of Disease - High-Grade / Low-Grade 3. Histological Features
27
What are the Features of a Low Grade Non-Hodgkin Lymphoma?
1. Indolent - Often Asymptomatic 2. Responds to Chemotherapy but Incurable 3. Median Survival Rate Varies by Subtype
28
What are the Features of a High Grade Non-Hodgkin Lymphoma?
1. Aggressive - Fast Growing 2. Require Combination Chemotherapy 3. Can be Cured, but Varies Widely
29
What are the Specific Disease Entities of Non-Hodgkin Lymphoma?
1. Diffuse Large B-Cell Lymphoma - High Grade Lymphoma | 2. Follicular Lymphoma - Low Grade Lymphoma
30
What is the Treatment of Non-Hodgkin Lymphoma?
Combination Chemotherapy: 1. Anti-CD20 Monoclonal Antibody 2. Chemo
31
What is Hodgkin Lymphoma associated with?
1. Epstein Barr Virus 2. Familial Clustering 3. Geographical Clustering
32
What is the Treatment of Hodgkin Lymphoma?
1. Combination Chemotherapy 2. +/- Radiotherapy 3. Use of PET Scan to assess Response to Treatment