7.2. Haematological Malignancies - Lymphoid Malignancies Flashcards
What does the Multipotential Haematopoietic Stem Cell Divide into?
- Common Myeloid Progenitor
2. Common Lymphoid Progenitor
What does the Common Myeloid Progenitor (From the Multipotential Haematopoietic Stem Cell) divide into?
- Megakaryocyte - THROMBOCYTES
- ERYTHROCYTES
- MAST CELLS
- Myeloblast:
- a) BASOPHIL
- b) NEUTROPHIL
- c) EOSINOPHIL
- d) Monocyte - MACROPHAGE
What does the Common Lymphoid Progenitor (From the Multipotential Haematopoietic Stem Cell) divide into?
- NATURAL KILLER CELL (Large Granular Lymphocyte)
- Small Lymphocyte:
- a) T-LYMPHOCYTE
2 b) B-LYMPHOCYTE - c) PLASMA CELL
Note - The B-Lymphocyte goes on to form the Plasma Cell
What is the Difference between Leukaemia and Lymphoma?
These are Descriptive Terms or Presentation of the Same Disease:
- Leukaemia - Predominantly in the Bone Marrow / Blood
- Lymphoma - Predominantly in the Lymph Nodes / Solid Organs
What are the Major Groups of Haematological Malignancies?
- Acute Leukaemias
- Chronic Leukaemias
- Malignant Lymphomas
- Multiple Myeloma
- Myelodysplastic Syndromes (MDS)
- Chronic Myeloproliferative Diseases (Biologically Malignant)
What are the Types of Acute Leukaemias?
- Acute Lymphoblastic Leukaemia (ALL)
2. Acute Myeloid Leukaemia (AML)
What are the Types of Chronic Leukaemias?
- Chronic Lymphocytic Leukaemia
2. Chronic Myeloid Leukaemia
Where do the Major Groups of Haematological Malignancies occur?
Haemopoietic Stem Cell:
- Lymphoid Progenitor Cell - Acute Lymphblastic Leukaemia:
- a) B-Lymphocytes - Chronic Lymphocytic Leukaemia
- a) i) Geminal Centre - Lymphomas
- a) i) 1) Plasma Cells - Mulitple Myeloma
- b) T-Lymphocytes - Lymphomas
- Myeloid Progenitor Cell - Acute Myeloid Leukaemia
- a) Myeloproliferative Disorders
What is the Difference between Acute and Chronic Leukaemia?
Acute vs Chronic:
- No Differentiation vs Retained Ability to Differentiate
- Bone Marrow Failure vs Proliferation without Bone Marrow Failure
- Rapidly Fatal vs Survival of a Few Years if Untreated
- Curable vs Uncurable without BMT
What is the Difference Between:
- Hodgkin Lymphoma?
- Non-Hodgkin Lymphoma?
- Hodgkin Lymphoma is a Specific Disease
2. Non-Hodgkin Lymphoma is everything else - 50 subtypes
What are the Major Lymphoproliferative Disorders?
- Acute Lymphoblastic Leukaemia - Rare
- Chronic Lymphocytic Leukaemia
- Hodgkin Lymphoma
- High-Grade Non-Hodgkin Lymphoma - 1/3
- Low-Grade Non-Hodgkin Lymphoma - 1/3
What is Acute Lymphoblastic Leukaemia?
Neoblastic Disorder of Lymphocytes
What are the Features of Acute Lymphoblastic Leukaemia?
- 75% cases in children < 6 years old
- 75-90% are B-Cell Lineage
- Presents with a 2-3 week history of Bone Marrow Failure / Bone Pain / Joint Pain
What are the Laboratory Findings for Acute Lymphoblastic Leukaemia?
- Low Haemoglobin
- High White Cell Count
- Low Platelets
- > 20% B-Lymphocytes present in Bone Marrow
What is the Treatment of Acute Lymphoblastic Leukaemia?
- Chemotherapy
- Consolidation Therapy
- CNS Directed Treatment
Note - Maintenance Treatment for 18 months - Stem Cell Transplantation - if High Risk
What are the Poor Risk Factors for Acute Lymphoblastic Leukaemia?
- Increasing Age
- Increased White Cell Count
- Immunophenotype (more primitive forms)
- Cytogenetics / Molecular Genetics
- Slow / Poor Response to Treatment
What are the outcomes for:
- Adults with Acute Lymphoblastic Leukaemia?
- Children with Acute Lymphoblastic Leukaemia?
- a) Complete Remission Rate = 78-91%
- b) Leukaemia Free Survival at 5 years = 30-35%
- a) 5 year Overall Survival = 90%
- b) Poor Risk Patients 5 year = 45%
What is the Clinical Presentation of Chronic Lymphocytic Leukaemia?
- Asymptomatic
- Bone Marrow Failure - Anaemia / Thrombocytopenia
- Lymphadenopathy
- Hepato/Splenomegaly
- Fever / Sweats / Weight Loss
- Infections / Immune Paresis
- Haemolytic Anaemia
What are the Laboratory Findings for Chronic Lymphocytic Leukaemia?
- Blood > 5 x 10^9 / L Lymphocytes
- Bone Marrow > 30% Lymphocytes
- Characteristic Immunophenotyping - B-Cell Markers
What is the Staging Method used for Chronic Lymphocytic Leukaemia?
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
What are the Indications for Treatment of Chronic Lymphocytic Leukaemia?
- Progressive Bone Marrow Failure
- Massive Lymphadenopathy
- Progressive Splenomegaly
- Lymphocyte Doubling Time < 6 Months
- > 50% Lymphocyte Increase over 2 Months
- Systemic Symptoms
- Autoimmune Cytopenias
What is the Treatment of Chronic Lymphocytic Leukaemia?
- Watch and Wait
- Cytotoxic Chemotherapy - Fluarabine / Bendamustine
- Monoclonal Antibodies - Rituximab / Obinutuzamab
- Novel Agents - Tyrosine Kinase Inhibitor
- Bone Marrow Transplant
What are the Poor Prognostic Markers of Chronic Lymphocytic Leukaemia?
- Advanced Disease
- Atypical Lymphocyte Morphology
- Rapid Lymphocyte Doubling Time
- CD38+ Expression
- Loss / Mutation p53
What is the Clinical Presentation of a Lymphoma?
- Lymphadenopathy / Hepatosplenomegaly
- Extranodal Disease
- “B Symptoms”
- Bone Marrow Involvement
How is a Lymphoma Staged?
- Lymph Node Biopsy
- CT Scan
- Bone Marrow Aspirate and Trephine
What is a Non-Hodgkin Lymphoma Classified According to?
- Lineage - B-Cell / T-Cell
- Grade of Disease - High-Grade / Low-Grade
- Histological Features
What are the Features of a Low Grade Non-Hodgkin Lymphoma?
- Indolent - Often Asymptomatic
- Responds to Chemotherapy but Incurable
- Median Survival Rate Varies by Subtype
What are the Features of a High Grade Non-Hodgkin Lymphoma?
- Aggressive - Fast Growing
- Require Combination Chemotherapy
- Can be Cured, but Varies Widely
What are the Specific Disease Entities of Non-Hodgkin Lymphoma?
- Diffuse Large B-Cell Lymphoma - High Grade Lymphoma
2. Follicular Lymphoma - Low Grade Lymphoma
What is the Treatment of Non-Hodgkin Lymphoma?
Combination Chemotherapy:
- Anti-CD20 Monoclonal Antibody
- Chemo
What is Hodgkin Lymphoma associated with?
- Epstein Barr Virus
- Familial Clustering
- Geographical Clustering
What is the Treatment of Hodgkin Lymphoma?
- Combination Chemotherapy
- +/- Radiotherapy
- Use of PET Scan to assess Response to Treatment
