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ICC3 > myeloid disorders > Flashcards

myeloid disorders Flashcards

1
Q

what are the two types of myeloproliferative neoplasms ?

A

BCR-ABL +ve

BCR-ABL -ve

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2
Q

BCR-ABL +ve ?

A

chronic myeloid leukemia

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3
Q

at what stage are most patients diagnosed ?

A

at their chronic stage

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4
Q

what are the three phases of chronic myeloid leukemia ?

A

chronic phase
accelerated phase
blast phase ( worst prognosis)

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5
Q

what is the prognosis of each of the three stages of CML?

A

chronic phase - responsive to treatment
accelerated phased - decreased responsiveness
blast phase - resistant to treatment

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6
Q

what are the symptoms in the chronic phase of CML ?

A 
may be symptomatic OR 
fatigue 
weight loss 
abdominal pain or discomfort 
night sweats
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7
Q

accelerated phase symptoms ?

A

myelofibrosis

progressive splenomegaly

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8
Q

what is the treatment for CML ?

A

tyrosine kinase inhibitors (TKI) (Ruxolitinib)imatinib

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9
Q

what is a deep molecular response ?

A

reduction in BCR-ABL expression (MR 4-MR5)

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10
Q

what are the types of BCR-ABL -ve malignancies ?

A

there are the classic and non classic forms

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11
Q

what are the classic types of BCR-ABL -ve neoplasms ?

A 
essential thrombocythemia ( over production of platelets)
polycythemia vera (over production of RBCs)
primary myelofibrosis
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12
Q

what are the non classic types of BCR-ABL -ve neoplasms?

A

mastocytosis ( accumulation of mast cells)
chronic neutrophilic leukemia
chronic eosinophilic leukemia

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13
Q

what are the clinical complications of BCR-ABL -ve MPNs?

A

major thrombosis
splenomegaly
microvascular symptoms

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14
Q

how is essential thrombocythemia diagnosed ?

A

a platelete count of more than 450 for at least 2 months with no other cause

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15
Q

what are the risk factors of thrombocythemia ?

A

previous thrombosis

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16
Q

how would you treat essential thrombocythemia ?

A

acording to the risk

High risk: aspirin and hydroxyurea
intermediate risk: just aspirin
low risk : just aspirin

17
Q

how would you diagnose polycythemia vera?

A

hb >16.2 with no other cause

18
Q

what is the main cause of polycythemia vera ?

A

JAK-2 mutation

19
Q

what can you give a polycythemia vera patient ?

A

Hydroxyurea and aspirin may help
and venesection

20
Q

what is the problem with using hydroxyurea in Polycythemia vera ?

A

possible risk of transforming to AML

21
Q

what is myelofibrosis ?

A

replacement of bone marrow by fibrosis

22
Q

what are the clinical features of MF ?

A

anemia
splenomegaly
abnormal RBC

23
Q

what is the treatment for MF?

A

stem cell transplantation
blood transfusion
radiation therapy
splenectomy

24
Q

what are some preexisting disorders that may be accompanied with acute myeloid leukemia ?

A

myelodysplastic syndrome
fanconi’s anemia
down syndrome

25
Q

what kind of anemia is in fanconi’s anemia

A

aplastic anemia

26
Q

what are the clinical manifestations of acute myeloid leukemia ?

A

related to problems caused by bone marrow failure
leukemic cells infiltrating patients organs ( splenomegaly hepatomegaly, bone pain)
hyperurecemia
tumor lysis syndrome
coagulopathy

27
Q

what is tumor lysis syndrome ?

A 
a group of metabolic disturbances including :
Hyperkalemia
Hyperphosphataemia
Hyperuricaemia
Hypocalcaemia
28
Q

how is AML differentiated from ALL ?

A

positive sudan black and myeloperoxidase stain in AML

29
Q

what is present in bone marrow aspirate in AML ?

A

hypercellular
more than 20% blast cells
presence of Auer rods

30
Q

what are the methods used too diagnose AML

A

immunophenotyping
cytogenetics/FISH
Molecular genetics

31
Q

what are the prognostic parameters in AML

A

Age
Performance status poor
cytogenetics
response to induction chemotherapy

32
Q

what kind of supportive care should be offered for AML patients

A

transfusion
infection control
lymphopenia

33
Q

what is the purpose of combination therapy ?

A

reduce drug resistance
reduce drug toxicity
interrupt cell growth at multiple points in the cell cycle

34
Q

what to do after remission is achieved ?

A

intensification of therapy
high dose therapy
same drugs at higher doses

35
Q

what are the examples of BCR-ABL negative diseases ?

A

primary myelodysplasia
polycythemia vera

36
Q

what is the gene abnormality associated with CML ?

A

translocation of 9 22
t(9;22)
ph chromosome

37
Q

how is acute promyelocytic leukemia confirmed through cytogenetics ?

A

RARA gene
t(15;17)

38
Q
A

ICC3 (66 decks)

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