Acute Leukaemias, Pre-Leukaemic Conditions and Chronic Leukaemias Flashcards

1
Q

What is myelodysplastic syndrome (MDS) and how is it managed?

A
  • Clonal blood disorder characterised by failure of effective erythropoiesis (Low blood counts)
  • More common in the elderly and patients with previous chemotherapy or radiotherapy
  • Causes low levels of blood components in the myeloid line (i.e. anaemia, neutropenia, thrombocytopenia)
  • Increased risk of transforming into AML
  • May be asymptomatic or present with symptoms of anaemia, neutropenia or thrombocytopenia.
  • Diagnosis:
    • FBC
    • Blasts on blood film
    • Bone marrow aspiration and biopsy for diagnosis
  • Management:
    • Watchful waiting
    • Supportive treatment with blood transfusions
    • Chemotherapy
    • SCT
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2
Q

What are myeoproliferative disorders (MPDs) and how are they managed?

A
  • Uncontrolled proliferation of a single type of stem cell
  • Systemic symptoms include:
    • Fatigue
    • Weight loss
    • Night sweats
    • Fever
  • Main types include:
    • Essential thrombocythaemia (↑ platelets)
    • Polycythaemia vera (↑ RBCs/platelets/WBCs)
    • Myelofibrosis (↑ fibrous tissue/platelets/WBCs)
  • Clonal blood disorders where the JAK2, MPL and CALR genesare present
  • Diagnosis through bone marrow biopsy and testing for JAK2, MPL and CALR genes
  • Management of myelofibrosis:
    • Monitoring if mild symptoms
    • Allogenic SCT
    • Chemotherapy
    • Supportive management
  • Management of polycythaemia vera:
    • Venesection
    • Aspirin (reduce risk of clot formation)
    • Chemotherapy
  • Management of essential thrombocytopenia:
    • Aspirin (reduce risk of clot formation)
    • Chemotherapy
  • Risk of transformation to AML.
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3
Q

Acute Myeloid Leukaemia (AML)

A
  • Most common acute leukaemia in adults
  • Can be result of transformation of MPD
  • Symptoms include:
    • Lethargy
    • Infection
    • Bleeding and bruising
    • Bone pain
    • Gum swelling
    • Lymphadenopathy
    • Skin rash
  • Investigations will show:
    • Anaemia
    • Neutropenia
    • Thrombocytopenia
    • Blasts cells on blood film that can have rods in their cytoplasm called auer rods
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4
Q

Acute Lymphoid Leukaemia (ALL)

A
  • Most common leukaemia in children - the limping child
  • Associated with Down syndrome
  • Acute proliferation of a single type of lymphocyte - usually B lymphocytes, which replace other types of cells leading to pancytopenia
  • Presents with:
    • Purpuric rash
    • Unexplained bone pain
    • Lumps vs liquid (lymphoblastic lymphoma vs true ALL)
  • Blood film will show blast cells.
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5
Q

Chronic Myeloid Leukaemia (CML)

A
  • Three phases:
    • Chronic phase - lasts around 5 years, asymptomatic, raised WCC
    • Accelerated phase - abnormal blast cells take up high proportion of bone marow and patients become symptomatic
    • Blast phase - even higher proportion of blast cells (>30%), severe symptoms and often fatal
  • Presentation:
    • May be asymptomatic
    • Fatigue
    • Weight loss
    • Night sweats
    • Abdominal discomfort
    • Splenomegaly
  • Cytogenetic change is the Philadelphia chromosome (translocation between chromosome 9 and 22 - BRC-ABL)
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6
Q

Management of leukaemia

A
  • Primarily treated with chemotherapy and steroids
  • CML also uses Imatinib - blocks downstream target of phosphorylation]
  • CLL also uses alkylating agents, purine nucleosides and chemoimmunotherapy
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7
Q

Chronic Lymphocytic Leukaemia (CLL)

A
  • Most common leukaemia in adults overall
  • Chronic proliferation of single type of well differentiated lymphocytes - usually B lymphocytes
  • Often asymptomatic but can present with warm AHIA
  • CLL can transform into high-grade lymphoma called Richter’s transformation
  • Blood film shows smear or smudge cells
  • CD 5, 19, 20, 23 positive
  • Bone marrow shows nodular or diffuse infiltrate
  • Flow cytometry also used
  • Presentation:
    • May be asymptomatic
    • Lethargy
    • Night sweats
    • Weight loss
    • Symptoms of anaemia
    • Lymphadenopathy
    • Infection
  • Staging uses BINET
    • A <3 involved nodes (10yrs)
    • B > 3 involved nodes, liver, spleen (7yrs)
    • C - anaemia or thrombocytopenia (2 yrs)
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8
Q

Ages of presentation for leukaemia (remember ALL CeLLmates have CoMmon AMbitions)

A
  • Under 5 and over 45 – acute lymphoblastic leukaemia (ALL)
  • Over 55 – chronic lymphocytic leukaemia (CeLLmates)
  • Over 65 – chronic myeloid leukaemia (CoMmon)
  • Over 75 – acute myeloid leukaemia (AMbitions)
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9
Q

Tumour lysis syndrome

A
  • Caused by release of uric acid from cells being destroyed by chemotherapy
  • Can form crystals in intestinal tissue and tubules of kidneys leading to AKI
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