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HAEMOTOLOGY > MedEd Lecture 1 > Flashcards

MedEd Lecture 1 Flashcards

1
Q

From which cell does acute/chronic myeloid leukaemia originate from?

A

common myeloid progenitor or multipotential haematopoietic stem cell

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2
Q

From which cell does acute lymphoblastic leukaemia originate from?

A

common lymphoid progenitor

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3
Q

From which cells does chronic lymphoblastic leukaemia originate from?

A

small lymphocyte, T lymphocytes or B lymphocytes

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4
Q

What is the outcome of acute leukaemias?

A

rapid proliferation of malignant cells resulting in bone marrow failure - stops producing normal cells

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5
Q

What are the clinical manifestations of acute leukaemias?

A

anaemia, thrombocytopenia, leukopenia/neutropenia

  • bone pain
  • usually generally unwell or septic
  • less common is spelnomegaly due to acute onset
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6
Q

What are the clinical manifestations of chronic leukaemias?

A

slower proliferation of malignant cells

  • bone marrow can still produce normal cells at the start
  • clonal cells can pool in lymph nodes or in the spleen
  • lymphadenopathy and splenomegaly
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7
Q

Typical history for Acute lymphoblastic leukaemia

A
  • 2-5 year old child
  • hepatosplenomegaly
  • bone pain / limp
  • fevers
  • CNS symptoms
  • testicular swelling (rare but specific - due to pooling of the cells)
  • in adults it’s similar to AML but you get lymphadenopathy or thymic masses
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8
Q

Acute lymphoblastic leukaemia typical bloods

A

neutropenia, thrombocytopenia, anaemia

  • may have high white cell count
  • circulating blasts in blood (this is variable depending on progression - higher in marrow at start then blood later on)
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9
Q

What does Acute Lymphoblastic look like on a blood film?

A

High nucelus:cytoplasm ratio

Nucelus looks more ‘open’ - paler purple/pink

May have nucleoli which look like small white punched out areas

‘Hand mirrors’

  • usually impossible to distinguish ALL from AML on film
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10
Q

How is Acute Lymphoblastic usually diagnosed?

A

Bone marrow and flow cytometry

  • Shows TdT+
  • CD19/CD22 B cells
  • CD2/CD3/CD4/8 T cells
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11
Q

What mutation is associated with 20-30% of Acute lymphoblastic leukaemia in adults?

A

BCR-ABL1 t(9;22) (philadelphia chromosome)

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12
Q

How is acute lymphoblastic leukaemia managed?

A

Chemo until remission or transplant

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13
Q

Typical patient with Acute Myeloid leukaemia

A
  • older patients (unusual in kids)
  • pre-existing myelodysplastic syndrome
  • symptoms of cytopenias
  • come in with sepsis
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14
Q

Blood tests of patient with acute myeloid leukaemia

A

anaemia
leukocytosis
thrombocytopenia
neutropenia
lots of blast cells
- normal INR, abnormal could be due to DIC due to acute promyelotic leukaemia

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15
Q

What is acute promyelotic leukaemia and how is it treated

A

associated with Acute myeloid and is when you get DIC

  • come in with big bleeds as well
  • good prognosis if treated early
  • give All-Trans Retinoid Acid (forces the cells to differentiate, stops proliferation)
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16
Q

Acute myeloid leukoaemia on blood film

A
  • auer rods (one is enough to diagnose)
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17
Q

How would you diagnose Acute myeloid leukaemia?

A
  • look for auer rods
  • if none then flow cytometry:
    MPO and CD13/33 are expressed
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18
Q

What translocation is associated with acute promyelotic leukaemia?

A

T(15;17)

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19
Q

How is acute myeloid leukaemia treated?

A

chemo

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20
Q

What are myeloproliferative neoplasms?

A

when you’re making too much of the cells of the myeloid lineage

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21
Q

What is essential thrombocythaemia?

A

Making too many platelets

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22
Q

How many platelets in essential thrombocythaemia?

A

over 450 times 10 to the 9

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23
Q

What mutation is associated with essential thrombocythaemia?

A

JAK2 (55% of cases)

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24
Q

How is essential thrombocythaemia treated?

A
  • aspirin to reduce stroke risk
  • hydroxycarbamaide lowers the count
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25
What is the haematocrit in polycythaemia vera?
over 0.52 in men over 0.48 in women
26
Complications of polycythaemia vera
You also get thrombocythaemia so high risk of thrombotic events - can become myelofibrosis
27
Mutation for polycythaemia vera
JAK2 (95%)
28
Treatment for polycythaemia vera
aspirin for stroke risk - venesection to lower haematocrit - hydroxycarbamide to lower count
29
What is myelofibrosis?
clonal proliferation of stem cells in the bone marrow resulting in cytokine release and fibrosis of the bone marrow - therefore reduced production of all cell lineages due to the fibrosis
30
Associations with myelofibrosis
JAK2 (50%) Pancytopenia Splenomegaly (massive)
31
Management of myelofibrosis
- stem cell transplant - Ruloxitnib - JAK inhibitor
32
What happens in Chronic myeloid leukaemia?
overproduction of myeloid lineage (so it's a type of myeloproliferative neoplasm)
33
Typical presentation of chronic myeloid leukaemia
35-55 age LUQ pain - splenomegaly Usually asymptomatic during chronic phase - May present with symptoms of acute leukaemia in the accelerated/blast phase
34
Bloods in chronic myeloid leukaemia
- leukocytosis - neutrophilia - platelets high initially - low monocytes - high basophils
35
Blood film in chronic myeloid leukaemia
' left shift ' leukocytosis eosinophilia basophilia
36
What chromosome is Chronic myeloid leukaemia associated with?
Philadelphia = BCR-ABL1 due to t(9;22)
37
What are the phases of chronic myeloid leukaemia?
Chronic Accelerated (increasing blasts, lack of response to therapy) Blast phase (over 20% of bone marrow cells are blasts, behaves like an acute leukaemia)
38
How is chronic myeloid leukaemia treated?
Tyrosine kinase inhibitor 1 = Imatinib over 90% 10 year survival
39
Chronic lymphoblastic leukaemia presentation
- usually asymptomatic - picked up on routine bloods - usually over 50 with increasing incidence with age - more common in men - lymphadenopathy/splenomegaly - can present with ITP/haemolytic anaemia
40
Blood and blood film for chronic lymphoblastic leukaemia
normal except raised white cell count (predominantly mature lymphocytes) smear/smudge cells
41
How is chronic lymphoblastic leukaemia diagnosed?
- smear/smudge cells - flow cytometry = ' clonal ' population of cells will express same cell markers (one of kappa or lambda light chains), generally B cell markers
42
How is chronic lymphoblastic leukaemia treated?
Stage A = no cytopenia, <3 areas of lymphoid involvement = watch and wait Stage B = no cytopenia, 3+ areas of lymphoid involvement = consider treatement Stage C = cytopenia = treat (targeted drugs)
43
What is Richters syndrome?
when CLL transforms into an aggressive acute leukaemia
44
What are myelodysplastic syndromes?
dysplastic changes (abnormal) to one or more myeloid cell lines (erythroid, megakaryocyte, granulocyte) - usually asymptomatic but can progress to AML - incidental cytopenia
45
Summary of difference between Hodgkins and Non-Hodgkins
Hodgkins = young, mediastinum pymph nodes, agressive, mostly curable Non-Hodgkins = older, lymph nodes anywhere, variable course and varibale curability
46
Staging of lymphoma
Ann-Arbor staging Stage 1 = Nodes affected on one group Stage 2 = More than one group but on same side of diaphragm Stage 3 = Gone to both sides of diaphragm Stage 4 = bone marrow/spleen involvement
47
B Symptoms of lymphoma
fever over 38 drenching night sweats unintentional weight loss over 10% in past 6 months
48
Hodgkin's summary
young people B symptoms mediastinal lymphadenopathy Redd-Sternburg cells are diagnostic (2 nuclei) associated with EBV treatment is chemo + radio
49
Non-Hodgkin's summary
lots of types usually B cell B symptoms or lymphadenopathy incidence increases with age
50
Examples of indolent non-hodgkin's lymphoma
Follicular lymphoma Small lymphocytic lymphoma
51
Follicular lymphoma genetics and diagnosis
t(14;18) causes fusion of BCL2 gene centroblasts on lymph node biopsy
52
Follicular lymphoma treatment
watch and wait unless high burden of disease
53
Example of a high-grade non-hodgkin's
diffuse large B-cell lymphoma
54
Risk of what with diffuse large B cell lymphoma treatment and Burkitt's lymphoma
tumour lysis syndrome
55
What is mantle cell lymphoma?
t(11;14) causes overexpression of cyclin D1 - treat with chemo
56
Example of very high grade lymphoma
Burkitt's
57
Associations, genetics and presentation of Burkitt's
' starry sky' on histology associated with t(8;14) - associated with EBV and HIV - large, fast growing lymph nodes in neck and elsewhere
58
Give an example of a T cell lymphoma and its associations
Adult T cell lymphoma - HTLV-1 common in Japan - ' flower cells' on blood film
59
What is multiple myeloma?
clonal population of plasma cells which proliferate and produce monoclonal immunoglobulin light chains
60
Key diagnosis findings for multiple myeloma
Blood = paraprotein IgG or IgA Urine = Bence Jones Protein
61
How is myeloma define clinically?
clonal bone marrow plasma cells account for over 10% of the bone marrow AND end organ damage (CRAB) 1 or more of Calcium over 2.75 Renal: creatinine clearance less than 40ml/min / creatinine over 177 Anaemia: Hb less than 100g/l Bone lesions - lytic lesions
62
What is smouldering myeloma
thing before multiple myeloma where you have high plasma and/or bence jones or paraprotein cells over 30 but don't have any organ damage yet
63
What is MGUS?
before multiple myeloma where you're producing the paraprotein but that's it, paraprotein still under 30 tho
64
Treatment of myeloma
MGUS = annual blood test Smouldering / myeloma = treat - Urine bence jones - whole body imaging to rule out lytic lesions - Stem cell transplant - targeted drugs
65
What is Waldenstrom's macrogloublinaemia
like myeloma but IgM is raised and not IgG or IgA

HAEMOTOLOGY (3 decks)

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