Chronic Leukaemias Flashcards
What is the commonest leukaemia?
Chronic lymphocytic leukaemia
What are risk factors for CLL?
- Trisomies e.g. Down’s syndrome
- Del17p13 (deletions)
What are red flag symptoms of CML?
- Night sweats
- Weight loss
- Fever
- Fatigue
What are symptoms of CLL?
- Often asymptomatic
- May be anaemic, infection-prone or have weight loss, sweats, anorexia if severe
What are signs of CLL?
- Lymphadenopathy - enlarged, rubbery, non tender nodes
- Hepatomegaly
- Splenomegaly
What will you see on blood tests in CLL?
- Low platelets
- Haemolytic anaemia (autoimmune)
- Increased lymphocytes
- Decreased neutrophils
What would you see on blood film in CLL?
Smudge/smear cells
How do you diagnose CLL & CML?
BM biopsy
Which infection can be commonly seen in CLL?
Herpes zoster
What are complications of CLL?
- Diffuse large B cell lymphoma (DLBL) - aggressive lymphoma (Richter’s syndrome)
- Autoimmune haemolysis
- Increased infection due to hypogammaglobulinaemia (low IgG)
- Marrow failure
What staging is used in CLL?
Rai staging
What other investigation can you do in all leukaemias?
Immunophenotyping + molecular/cytogenic analysis
What is the first line treatment for CLL?
Chemo + targeted - FCR (fludarabine, cyclophosphamide, rituximab) OR venetoclax ± steroids ± radiotherapy ± stem cell transplantation
What happens in chronic leukaemias?
- Cells mature partly but not functioning completely
- Not as obvious as acute - cells can look quite normal but they are not
- Cells survive longer than normal cells so they gradually crowd out normal cells - harder to fight infection
How is the onset and progression of CLL?
Insidious gradual onset - people can live with it for years
What is the hallmark of CLL?
Progressive accumulation of a malignant clone of functionally incompetent cells
What type of patients get CML?
40-60 years, male predominance
What is the main risk factor for CML present in > 80% of patients?
Philadelphia chromosome (BCR-ABL gene)
What is CML?
Uncontrolled clonal proliferation of myeloid (granulocytic) cells at different stages of differentiation
What are symptoms of CML?
- Chronic & insidious - weight loss, night swears, fatigue, fever
- Features of gout (due to purine breakdown)
- Bleeding (platelet dysfunction)
- Abdominal discomfort (splenic enlargement)
- 30% detected by change
What are the signs of CML?
- Splenomegaly (> 75%) - often massive
- Hepatomegaly
- Anaemia
- Bruising
What is a complication of CML?
Progression to AML (blast crisis/transformation)
How do you treat CML?
Imatinib (BCR-ABL tyrosine kinase inhibitor) - targeted ± BMT
What would you see on blood tests for CML?
- Increased WCC (often > 100) (myeloid cells)
- Low or normal Hb
- Platelets variable
- Increased urate
- Increased B12
What would you cytogenic analyse for Philadelphia chromosome?
Blood or bone marrow
What are the 3 myeloid neoplasms (clonal disorders of haematopoeisis)?
1) Myeloproliferative neoplasms
2) Myelodysplastic syndrome
3) Acute myeloid leukaemia
What is leukocytosis?
Lots of blast cells in the bone marrow
What is the key feature of leukaemia?
Leukocytosis
What is myeloproliferative neoplasms?
- Too many morphologically normal myeloid cells - uncontrolled growth of cells derived from myeloid lineage i.e. RBCs, platelets, innate immune cells
- These are mature cells so their disease course is typically chronic
- i.e. polycythaemia e.g. polycythaemia rubra vera
What is myelodysplastic syndrome?
Morphologically abnormal cells due to issues with maturation down the myeloid pathway
What are risk factors for myelodysplastic syndrome?
- Smoking
- Chemotherapy
- Lead and mercury exposure
What can myelodysplastic syndrome develop into?
Acute myeloid leukaemia
What is polycythaemia rubra vera an example of?
Myeloproliferative neoplasm
What age group typically get CLL?
Elderly
