7.1. Haematological Malignancies - Introduction and Myeloid Malignancies Flashcards
What is the Epidemiology of Haematological Malignancies?
- 10% of All Human Cancers
- Occur In All Age Groups - Peak incidence of Hodgkin Lymphoma = 18-35 vs Myelodysplastic Syndromes = 80’s
- Males more commonly affected than Females
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 are the indications of Lymphadenopathy being:
- Localised and Painful?
- Localised and Painless?
- Generalised and Painful / Tender?
- Generalised and Painless?
- Bacterial Infection in Draining Site
- Metastatic Carcinoma / Lymphoma / Rare Infections / Reactive (no cause identified)
- Viral Infections - EBV / CMV / Hepatitis / HIV
- Lymphoma / Leukaemia / Connective Tissue Disease / Drugs / Reactive (no cause identified)
What are the Clinical Features of a Multiple Myeloma (Plasma Cell Malignancy)?
- Bone Pain and Lytic Lesions
- Anaemia
- Recurrent Infetcion
- Renal Failure
- Amyloidosis
- Bleeding Tendency
- Hyperviscocity Syndrome
What are the Clinical Features of Acute Myeloid Leukaemia?
- Bone Marrow Failure
- Anaemia
- Thrombocytopenic Bleeding - Purpura / Mucosal Membrane
- Infection because of Neutropenia - Bacterial / Fungal
What are the Essential Investigations for Acute Myeloid Leukaemia?
- Blood Count / Blood Film
- Bone Marrow Aspirate / Trephine
- Cytogenetics of Leukaemic Blasts
- Immunophenotyping of Leukaemic Blasts
- CSF Examination, if Symptomatic
What is the Treatment of Acute Myeloid Leukaemia?
- Supportive Care
- Anti-Leukaemic Chemotherapy
- Stem Cell Transplantation - Allogenic
- All-Trans Retinoic Acid - in Acute Promyelocytic Leukemia
What are the Clinical Features of Chronic Myeloid Leukaemia?
- Anaemia
- Splenomegaly - often Massive
- Weight Loss
- Hyperleukostasis - Fundal Haemorrhage / Venous Congestion / Altered Consciousness / Respiratory Failure
- Gout
What are the Laboratory Features of Chronic Myeloid Leukaemia?
- High White Cell Count
- High Platelet Count
- Anaemia
- Blood Film shows all stages of White Cell Differentiation with Increased Basophils
- Bone Marrow is Hypercellular
- Bone Marrow and Blood Cells contain the Philadelphia Chromosome
What is the Treatment of Chronic Myeloid Leukaemia?
- Tyrosine Kinase Inhibitors - Imatinib / Dasatinib / Nilotinib
- Allogenic Transplantation - if above fails
What are the Features of Myelodysplastic Syndromes?
- Acquired Clonal Disorders of the Bone Marrow
- Commonly seen in old age
- Fatal as a Result of Pregression to Bone Marrow Failure / AML
What is the Presentation of Myelodysplastic Syndromes?
- Macrocytic Anaemia
- Pancytopenia
- Pre-Leukaemic
What is the Treatment of Myelodysplastic Syndromes?
- Supportive
2. Stem Cell Transplantation - for the young patients
What are the Common Myeloproliferative Diseases?
- Polycythaemia Vera
- Essential Thrombocythaemia
- Idiopathic Myelofibrosis
What are the Clinical Features of Polycythaemia Vera?
- Headaches
- Itch
- Vascular Occlusion
- Thrombosis
- TIA / Stroke
- Splenomegaly
What are the Laboratory Features of Polycythaemia Vera?
- Raised Haemoglobin Concentration / Haematocrit
- Raised WCC / Platelet Count
- Raised Uric Acid
- True increase in Erythrocyte Mass
What is the Treatment of Polycythaemia Vera?
- Venesection - to keep the Haematocrit Low
- Aspirin
- Hydroxcarbamide
What is the Natural History of Polycythaemia Vera?
- Stroke / Thrombus if poorly controlled
- Bone Marrow Failure - from the Secondary Myelofibrosis
- Transformation to AML
What are the Features of Essential Thrombocythaemia?
- Myeloproliferative Disease with the Predominant Feature of Raised Platelet Count
- Arterial / Venous Thromboses
- Digital Ischaemia
- Gout
- Mild Splenomegaly
Note - Can Progress to Myelofibrosis / AML
What is the Treaatment of Essential Thrombocythaemia?
- Aspirin
2. Hydroyurea