Haematological Malignancies Flashcards
What is the epidemiology of haematological malignancies?
- Haematological Malignancies account for approximately 10% of all human cancers
- They occur in all age groups, including children
- Adult males are more commonly affected than females
What is the age distribution of new Hodgekin lymphoma cases?
Increase in incidence between 20-35 and 70+
What is the age distribution of Non-Hodgkin lymphoma?
Increases with age with peak between 70 and 85
What is the pathogenesis of haematological malignancy?
- Multi step process
- Result of acquired genetic alterations to long lived cells
- Proliferative/survival advantage to that mutated cell
- Production of malignant clone
- Malignant clone grows to dominate the tissue
What are the properties of stem cells?
- Able to self-renew
- Able to differentiate
What is the origin of myeloid malignancies?
- RBC
- Platelets
- Granulocytes
- Monocytes
What is the origin of lymphoid malignancies?
- B-cell
- T-cell
What type of cancer arises from myeloid progenitor cells?
Acute myeloid leukaemia
What type of cancer arises from lymphoid progenitor cells?
Acute lymphoblastic leukaemia
What occurs in acute myeloid leukaemia?
- Ongoing proliferation
- Failure to differentiate
What occurs in myeloproliferative disorders?
- Ongoing proliferation at a high rate
- Ongoing differentiation
What occurs in acute lymphoblastic leukaemia?
Proliferation without differentiation
What type of cancer arises from WBC, platelets and RBCs?
Myeloproliferative disorder
Leukaemia vs Lymphoma
Descriptive terms related to distribution of disease
- Leukaemia is in the blood and bone marrow
- Lymphomas is in the lymph glands and other solid tissues
What are the major groups of haematological malignancies?
- Acute leukaemias
- Chronic leukaemias
- Malignant lymphomas
- Multiple myeloma
- Myelodysplastic syndromes
- Chronic myeloproliferative neoplasms
Name the 2 types of acute leukaemia
- Acute lymphoblastic leukaemia
- Acute myeloid leukaemia
Name the 2 types of chronic leukaemia
- Chronic myeloid leukaemia
- -Chronic lymphocytic leukaemia
Name the 2 types of malignant lymphomas
- Non-Hodgekin lymphoma
- Hodgekin Lymphoma
What are the clinical features of acute leukaemia?
Triad of bone marrow failure
- Anaemia
- Thrombocytopenic bleeding (purpura and mucosal membrane bleeding)
- Infection because of neutropenia (predominantly bacterial and fugal)
Briefly describe B cell maturation
- Progenitor B cell> Pre B cell>Immature B cell in the bone marrow
- Naïve B cells migrate to the germinal centres of lymph node follicles where they are presented with antigens as centroblasts and undergo somatic hypermutation to form centrocytes
- They can mature into memory B cells or plasma cells
How do lymphomas present?
- Lymphadenopathy
- > 90% HL present with nodal disease
- 60% NHL present with purely nodal disease
- Extranodal disease
- 40% of NHL present with an extranodal component
- Systemic symptoms
- Fever, drenching sweats, loss of weight, pruritus, fatigue
What does localised and painful lymphadenopathy suggest?
Bacterial infection in draining site
What does localised and painless lymphadenopathy suggest?
- Rare infections, catch scratch fever, TB
- Metastatic carcinoma from draining site - hard
- Lymphoma - rubbery
- Reactive, no cause identified
What does generalised and painful/tender lymphadenopathy suggest?
Viral infections like EBV, CMV, hepatitis and HIV
What does generalised and painless lymphadenopathy suggest?
- Lymphoma
- Leukaemia
- Connective tissue disease, sarcoidosis
- Reactive, no cause identified
- Drugs
Acute leukaemia vs chronic leukaemia
Do leukaemic cells differentiate?
Acute leukaemia - Leukaemic cells do not differentiate
Chronic leukaemia - Leukaemic cells retain ability to differentiate
Acute leukaemia vs chronic leukaemia
Bone marrow
Acute leukaemia - Bone marrow failure
Chronic leukaemia - Proliferation without bone marrow failure
Acute leukaemia vs chronic leukaemia
Survival
Acute leukaemia - Rapidly fatal if untreated
Chronic leukaemia - Survival for a few years
Acute leukaemia vs chronic leukaemia
Curable?
Acute leukaemia - Potentially curable
Chronic leukaemia - Potentially curable with modern therapy eg Tyrosine Kinase inhibitors in CML
