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Clinical Pathology > Chronic myeoproliferative disorders and chronic myeloid leukaemia. > Flashcards

Chronic myeoproliferative disorders and chronic myeloid leukaemia. Flashcards

1
Q

define chronic myeloproliferative disorders.

A

long term myeloid lineage cells hyper proliferate.

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2
Q

what blood cancer can chronic myeloproliferative disorders progress too

A

leukaemia

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3
Q

what cells are contained within stem cells

A

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

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4
Q

define polycythaemia vera

A

increased RBC, +/- neutrophils and platelets.

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5
Q

deine essential thrombocthaemia

A

increased platelets

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6
Q

define myelofibrosis

A

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

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7
Q

3 myleoprolifertive disorders

A

myelofibrosis
polycathaemia vera
essential thrombocythaemia.

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8
Q

symptoms of polycythaemia vera

A

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

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9
Q

how is polycathaemia vera diagnosed

A

Persistent increased Hb/hct (haemoatocrit) >0.5

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10
Q

what is the difference between relative and absolute polycathemia.

A

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.

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11
Q

what is the difference between primary and secondary polycythaemia.

A

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

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12
Q

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

A

FBC
Ferritin
EPO level
UE/LFT

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13
Q

what is the only primary cause of polycythaemia

A

polycythaemia vera

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14
Q

what are the secondary causes of polycythaemia.

A 
central hypoxic process
renal disease -EPO
drug associated- androgen preparations
Congenital- high oxygen affinity, erthythrpoeitien receptor mediated.
Idiopathic erythrocytosis
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15
Q

what are the second line tests for polycthaemia

A

CXR-chest xray
ABG
USS-abdomen for spleenomegaly.

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

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16
Q

what change takes place in the JAK2 mutation.

A

G-to-T mutation at nucleotide 1849

Phenylalanine for valine at 617 in protein (V617F)

17
Q

how does the JAK2 mutation cause myeloproliferative disorders

A

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

18
Q

Treatment for polycythaemia vera

A

venesection (remove RBC) aim to get haemocrit 0.45.

Aspirin.

19
Q

causes of reactive (secondary) thrombocytosis

A

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

20
Q

what are the first line investigations of thrombocytosis

A
  1. FBC and film
  2. Ferritin
  3. CRP
  4. CXR
  5. ESR
21
Q

what are the second line investigations for thrombocytosis

A
  • JAK2
  • CALR
  • ? Bone marrow biopsy
  • Extensive search for secondary cause
22
Q

what is CALR

A

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

23
Q

treatment for thrombocytosis

A

Aspirin 75mg daily- inhibits platelets.

Cytoreduction (only if High risk)

24
Q

what is cytorecustion (used in thrombocytosis treatment)

A

Hydroxycarbamide- antifolate- reduced DNA synthesis.

25
Q

Clinical presentation of myelofibrosis.

A

pancytopenia- deficney in all 3 blood cell types

fever, night sweats and weight loss.

26
Q

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

A

polycathemia and myelofibrosis

27
Q

what are the first line investigations for myelofibrosis

A

FBC and film

28
Q

how is myelofibrosis diagnosed

A

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

29
Q

how do you measure spleen size

A

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

30
Q

causes of spleenomegaly

A 
Cancer
Hameatological -myelofibrosis.
Infection
Congestion-liver/portal disease
Autoimmune
Glycogen storage disorders
Other- amyloid.
31
Q

Treatment for myelofibrosis

A

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

32
Q

which of the 3 myeloporliferative disorders has poor prognosis

A

myelofibrosis

33
Q

what are the clinical features of chronic myeloid leukaemia

A

leukocytosis- lots of WBC.
anaemia.
splenomegaly.

34
Q

symptoms of chronic myeloid leukaemia

A
  • Abdominal discomfort:Splenomegaly
  • Abdominal pain: Splenic infarction
  • Fatigue: Anaemia, catabolic state
  • Venous occlusion: Retinal vein, DVT, priapism (continuous erection cause infract)
  • Gout- Hyperuricaemia
35
Q

treatment for chronic myeloid leukaemia.

chronic and acute phase

A

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

Acute transformation- intensive chemotherapy

Allogenic bone marrow transplant

36
Q

What common translocation increases the chance of developing chronic myeloid leukaemia

A
  • 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
Q

which drug inhibits the Philadelphia chromosome

A

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

38
Q

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

A

-Imatinib.

39
Q

what are the 2 phases of chronic myeloid leukaemia

A

chronic

acute transformation.

Clinical Pathology (45 decks)

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