myeloid disorders

Question 1

what are the two types of myeloproliferative neoplasms ?

Answer 1

BCR-ABL +ve

BCR-ABL -ve

Question 2

BCR-ABL +ve ?

Answer 2

chronic myeloid leukemia

Question 3

at what stage are most patients diagnosed ?

Answer 3

at their chronic stage

Question 4

what are the three phases of chronic myeloid leukemia ?

Answer 4

chronic phase
accelerated phase
blast phase ( worst prognosis)

Question 5

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

Answer 5

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

Question 6

what are the symptoms in the chronic phase of CML ?

Answer 6

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

Question 7

accelerated phase symptoms ?

Answer 7

myelofibrosis

progressive splenomegaly

Question 8

what is the treatment for CML ?

Answer 8

tyrosine kinase inhibitors (TKI) (Ruxolitinib)imatinib

Question 9

what is a deep molecular response ?

Answer 9

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

Question 10

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

Answer 10

there are the classic and non classic forms

Question 11

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

Answer 11

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

Question 12

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

Answer 12

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

Question 13

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

Answer 13

major thrombosis
splenomegaly
microvascular symptoms

Question 14

how is essential thrombocythemia diagnosed ?

Answer 14

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

Question 15

what are the risk factors of thrombocythemia ?

Answer 15

previous thrombosis

Question 16

how would you treat essential thrombocythemia ?

Answer 16

acording to the risk

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

Question 17

how would you diagnose polycythemia vera?

Answer 17

hb >16.2 with no other cause

Question 18

what is the main cause of polycythemia vera ?

Answer 18

JAK-2 mutation

Question 19

what can you give a polycythemia vera patient ?

Answer 19

Hydroxyurea and aspirin may help
and venesection

Question 20

what is the problem with using hydroxyurea in Polycythemia vera ?

Answer 20

possible risk of transforming to AML

Question 21

what is myelofibrosis ?

Answer 21

replacement of bone marrow by fibrosis

Question 22

what are the clinical features of MF ?

Answer 22

anemia
splenomegaly
abnormal RBC

Question 23

what is the treatment for MF?

Answer 23

stem cell transplantation
blood transfusion
radiation therapy
splenectomy

Question 24

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

Answer 24

myelodysplastic syndrome
fanconi’s anemia
down syndrome

Question 25

what kind of anemia is in fanconi's anemia

Answer 25

aplastic anemia

Question 26

what are the clinical manifestations of acute myeloid leukemia ?

Answer 26

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

Question 27

what is tumor lysis syndrome ?

Answer 27

``` a group of metabolic disturbances including : Hyperkalemia Hyperphosphataemia Hyperuricaemia Hypocalcaemia ```

Question 28

how is AML differentiated from ALL ?

Answer 28

positive sudan black and myeloperoxidase stain in AML

Question 29

what is present in bone marrow aspirate in AML ?

Answer 29

hypercellular more than 20% blast cells presence of Auer rods

Question 30

what are the methods used too diagnose AML

Answer 30

immunophenotyping cytogenetics/FISH Molecular genetics

Question 31

what are the prognostic parameters in AML

Answer 31

Age Performance status poor cytogenetics response to induction chemotherapy

Question 32

what kind of supportive care should be offered for AML patients

Answer 32

transfusion infection control lymphopenia

Question 33

what is the purpose of combination therapy ?

Answer 33

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

Question 34

what to do after remission is achieved ?

Answer 34

intensification of therapy high dose therapy same drugs at higher doses

Question 35

what are the examples of BCR-ABL negative diseases ?

Answer 35

primary myelodysplasia polycythemia vera

Question 36

what is the gene abnormality associated with CML ?

Answer 36

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

Question 37

how is acute promyelocytic leukemia confirmed through cytogenetics ?

Answer 37

RARA gene t(15;17)