Myelodysplastic Syndromes (MDS)and Acute Myeloid Leukaemia (AML Flashcards

1
Q

define myelodysplatic syndromes

A

myelo- marrow
dysplastic- abnormal or funny looking

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2
Q

describe myelodysplatic syndromes ( L)

A

-proportion of leukaemia cells or blasts <20% nucleated cels in bone marrow
=Bone marrow cells fail to make adequate numbers of healthy blood cells
i.e. both quantity and quality (function) of cells are affected
-Abnormal cells crowd out remaining normal cells

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3
Q

what is can myelodysplatic syndromes progress to?

A

acute myeloid leukaemia

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4
Q

What is Acute Myeloid Leukaemia?

A

Acute myeloid leukaemia (AML) is a heterogeneous clonal malignancy characterised by
-immature myeloid cell proliferation (defined as ≥20% “blasts”)
and
-bone marrow failure

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5
Q

describe the epidemiology of MDS

A

4.5 in 100,000
Median age at diagnosis = 76 years

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6
Q

describe the epidemiology of AML

A

less than 1% each year
age 85-89

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7
Q

what are the diagnostic features of MDS in a full blood count blood test? ( L)

A

Low blood counts
-red cells (symptoms include fatigue, shortness of breath, lightheadedness)
-white cells (symptoms include increased risk of frequent and/or severe infections)
-platelets (bleeding/ bruising)

-Peripheral blood film demonstrates dysplastic features (e.g. hypogranular neutrophils, platelet anisopoikilocytosis, blasts)

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8
Q

what are the diagnostic features of AML in a full blood count blood test?

A

White cell counts can be
-Low
-Normal
-High
-symptoms include increased risk of frequent and/or severe infections (because a large proportion of the white cells are abnormal)

-Other blood parameters are usually low
–red cells (symptoms include fatigue, shortness of breath, lightheadedness)
-platelets (symptoms of bleeding/ bruising)

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9
Q

What are the potential differential diagnoses

A

B12/ folate or mixed haematinic deficiency
Infection (e.g. retroviral disease, herpesvirus)
Medications
Autoimmune
Liver disease (e.g. cirrhosis)
post operative
severe sepsis- Blasts in peripheral blood usually associated with neutrophil

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10
Q

in a patient with suspected MDS/AML what investigations should you do?

A

-A good history
-Review of previous blood test results
—Was the FBC previously normal?
–Has the Hb/ WC/ neutrophils/ platelet counts been downward trending for some weeks/ months/ years?
-FBC and blood film
-Haematinics (B12, folate, ferritin)

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11
Q

when should you be concerned? ( L)

A

-There are blasts on peripheral blood
-The deterioration in FBC parameters is very rapid (days/ weeks)
-Have a low threshold for all other patients to repeat FBC in 1-2 weeks and tell them to ring if they notice any new symptoms (i.e. symptomatic anaemia, infection, bleeding/ bruising)

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12
Q

how do you diagnose MDS and AML

A

Blood tests (full blood count & blood film)
Bone marrow aspirate and trephine biopsy

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13
Q

What is morphology?

A

= the appearance of cells on slides

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14
Q

in morphology what are the requirements for myelodysplastic syndrome?

A

Requirement of 10% dysplasia in any cell line(s)
Blast % can be anywhere from 0 – 19%

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15
Q

in morphology what are the requirements for acute myeloid leukaemia syndrome?

A

minimum 20% blasts

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16
Q

define leukemia

A

malignant proliferation of haemopoietic cells

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17
Q

what history is relevant when diagnosing leukaemia ?

A

-Anaemia
—–Fatigue
——Shortness of breath
-Infection
—–Tonsillitis
—–Fevers/ rigors
-Thrombocytopenia
–Bruising
–Bleeding – mucosal usually
–Rash
Low haemoglobin
Low platelets
Very low neutrophils
Blast cells present with Auer rods = acute myeloid leukaemia

18
Q

what tests do you do/ what do you look at to diagnose patients?

A

-Blood tests (morphology, cytogenetics, minimal residual disease monitoring, molecular genetics, deep sequencing, multi-parameter flow cytometry)

-Bone marrow aspirate and trephine biopsy

-Increase in blasts >20% (acute leukaemia)

-Background abnormalities to suggest pre-existing bone marrow abnormalities
-Cytogenetics for prognosis
-Molecular genetics for prognosis
-Immunophenotyping

19
Q

describe the onset of Chronic myeloid leukaemia

A

slow

20
Q

describe Chronic myeloid leukaemia film

A

left shift+ basophilia

21
Q

describe the Chronic myeloid leukaemia WCC

A

in a FBC- high WCC

22
Q

what is a key diagnostic feature of Chronic myeloid leukaemia

A

Philadelphia chromosome

23
Q

what treatments are used in Chronic myeloid leukaemia

A

arget molecular therapy – tyrosine kinase inhibitors: Imatinib

AML treatment
Chemotherapy and supportive measures
Take into account – age, fitness, comorbidities, AML features, clinical trials

24
Q

what is the Most common paediatric malignancy?

A

Acute lymphoblastic leukaemia

25
Q

describe the acute presentation of Acute lymphoblastic leukaemia

A

bone marrow failure
organ infiltration (cns)

26
Q

how is Acute lymphoblastic leukaemia diagnosed?

A

Diagnosis – as for AML
philadelphia chromosome

27
Q

what does It mean if the Philadelphia chromosome is present?

A

poor prognosis

28
Q

describe the treatment phases

A

1.Induction
2.Consolidation
3.Delayed intensification
4.Maintenance

29
Q

what are all the treatments?

A
  • treatment phases
  • CNS directed therapy
  • Stem cell transplant
30
Q

what is the most common leukaemia?

A

Chronic lymphocytic leukaemia

31
Q

describe Chronic lymphocytic leukaemia

A
  • has a gradual accumulation of B lymphocytes
  • blood/bone marrow
  • lymph glands - including spleen
32
Q

how is Chronic lymphocytic leukaemia
usually found?

A

incidentally on FBC

33
Q

describe the epidemiology of Chronic lymphocytic leukaemia

A

Generally elderly but 20% <55yrs

34
Q

describe the clinical course of Chronic lymphocytic leukaemia

A
  • variable
  • progressive lymphadenopathy/ hepatosplenomegaly
  • auto immune- haemolysis, ITP
  • bone marrow failure- due to marrow replacement
35
Q

what treatment is done for Chronic lymphocytic leukaemia

A

Do nothing
Chemotherapy monoclonal antibodies
Targeted therapy
Bone marrow transplant

36
Q

what does chemotherapy do?

A

causes damage preferentially to rapidly dividing cells

37
Q

what is used for chemotherapy ?

A

Combinations of drugs are commonly used

38
Q

why does dosing of drugs have to be carefully managed in chemotherapy?

A

to optimise balance between:
-Destroying leukemic cells
-Not causing irreversible toxicity to other normal cells

39
Q

what are other things to consider pre- chemo?

A

supportive measures- Fertility cryopreservation
clinical trial availability

40
Q

what are the side effects of chemo ?

A

-Nausea
-Cytopenias
-Anaemia
-Neutropenia
-Low platelets and risk of bleeding/ -bruising
-Bystander organ damage (kidneys, liver, etc)
-Temporary hair loss