Chronic myeoproliferative disorders and chronic myeloid leukaemia. Flashcards Preview

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Flashcards in Chronic myeoproliferative disorders and chronic myeloid leukaemia. Deck (39):
1

define chronic myeloproliferative disorders.

long term myeloid lineage cells hyper proliferate.

2

what blood cancer can chronic myeloproliferative disorders progress too

leukaemia

3

what cells are contained within stem cells

erythrocytes
Granulocytes (neutrophil, eosinophil and basophil)
Megakaryocytes (platelets)

4

define polycythaemia vera

increased RBC, +/- neutrophils and platelets.

5

deine essential thrombocthaemia

increased platelets

6

define myelofibrosis

proliferation of an abnormal clone of hematopoietic stem cells in the bone marrow and other sites results in fibrosis, or the replacement of the marrow with scar tissue

7

3 myleoprolifertive disorders

myelofibrosis
polycathaemia vera
essential thrombocythaemia.

8

symptoms of polycythaemia vera

Insidious, itching, plethoric face, headache, general malaise, tinnitus, peptic ulcer, gout-uric acid crystals, gangrene of the toes, plethora, gangrene, engorged retinal veins, spleenomegaly.

9

how is polycathaemia vera diagnosed

Persistent increased Hb/hct (haemoatocrit) >0.5

10

what is the difference between relative and absolute polycathemia.

relative is where RBC appear high in comparison to plasma which has decreased in volume happens in dehydrated e.g. alcoholic)
absolute is where the level of RBC is down.

11

what is the difference between primary and secondary polycythaemia.

primary is in the bone marrow, secondary is due to another factor such as living in high altitudes,

12

what is the first line of tests if you suspect polycythaemia.

FBC
Ferritin
EPO level
UE/LFT

13

what is the only primary cause of polycythaemia

polycythaemia vera

14

what are the secondary causes of polycythaemia.

central hypoxic process
renal disease -EPO
drug associated- androgen preparations
Congenital- high oxygen affinity, erthythrpoeitien receptor mediated.
Idiopathic erythrocytosis

15

what are the second line tests for polycthaemia

CXR-chest xray
ABG
USS-abdomen for spleenomegaly.

EPO Normal- JAK2 mutation, Bone marrow examination, EXON 12 mutation.

16

what change takes place in the JAK2 mutation.

G-to-T mutation at nucleotide 1849
Phenylalanine for valine at 617 in protein (V617F)

17

how does the JAK2 mutation cause myeloproliferative disorders

EPO binds to receptor with JAK2 and phosphorylation causes DNA replication, but with mutation receptor in activated without EPO.

18

Treatment for polycythaemia vera

venesection (remove RBC) aim to get haemocrit 0.45.
Aspirin.

19

causes of reactive (secondary) thrombocytosis

surgery, infection, inflammation, malignancy, iron deficiency, hyposplenism, haemolysis, drug induced (steroids or adrenaline, TPO), rebound post chemo

20

what are the first line investigations of thrombocytosis

1. FBC and film
2. Ferritin
3. CRP
4. CXR
5. ESR

21

what are the second line investigations for thrombocytosis

• JAK2
• CALR
• ? Bone marrow biopsy
• Extensive search for secondary cause

22

what is CALR

• cell signalling protein produced in ER (endoplasmic Reticulin) and found in myeloid progenitors.

23

treatment for thrombocytosis

Aspirin 75mg daily- inhibits platelets.
Cytoreduction (only if High risk)

24

what is cytorecustion (used in thrombocytosis treatment)

Hydroxycarbamide- antifolate- reduced DNA synthesis.

25

Clinical presentation of myelofibrosis.

pancytopenia- deficney in all 3 blood cell types
fever, night sweats and weight loss.

26

spleenomegaly is a complication of which 2 of the 3 myeloproliferative disorders

polycathemia and myelofibrosis

27

what are the first line investigations for myelofibrosis

FBC and film

28

how is myelofibrosis diagnosed

blood film, bone marrow results, JAK mutation and CALR mutation.

29

how do you measure spleen size

from mid clavicular line at the costal margin to the furthest margin at which the spleen can be felt.

30

causes of spleenomegaly

Cancer
Hameatological -myelofibrosis.
Infection
Congestion-liver/portal disease
Autoimmune
Glycogen storage disorders
Other- amyloid.

31

Treatment for myelofibrosis

Supportive care- EPO injections. Anaemic (blood transfusion), platelets transfused.
JAK2 Inhibitors- helps shrink spleen.
Bone marrow Transplant

32

which of the 3 myeloporliferative disorders has poor prognosis

myelofibrosis

33

what are the clinical features of chronic myeloid leukaemia

leukocytosis- lots of WBC.
anaemia.
splenomegaly.

34

symptoms of chronic myeloid leukaemia

• Abdominal discomfort:Splenomegaly
• Abdominal pain: Splenic infarction
• Fatigue: Anaemia, catabolic state
• Venous occlusion: Retinal vein, DVT, priapism (continuous erection cause infract)
• Gout- Hyperuricaemia

35

treatment for chronic myeloid leukaemia.
(chronic and acute phase)

chronic phase- Low dose oral cytotoxic drugs (busulphan, hydroxycarbamide).

Acute transformation- intensive chemotherapy

Allogenic bone marrow transplant

36

What common translocation increases the chance of developing chronic myeloid leukaemia

• ABL- chromosome 9
• BCR- chromosome 22
• Translocation results in these 2 sitting next to each other- oncogene, which codes for tyrosine kinase tells cells to hyperprolifearte, which causes anaemia.

37

which drug inhibits the Philadelphia chromosome

• Gleevac (imantinib)- block the active site in the bcr-abl tyrosine kinase.

38

BCR-ABL mutations confer resistance to which drug used to treat chronic myeloid leukaemia

-Imatinib.

39

what are the 2 phases of chronic myeloid leukaemia

chronic
acute transformation.