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Pathology Summaries > Haem: Myeloproliferative, myelodysplastic etc > Flashcards

Haem: Myeloproliferative, myelodysplastic etc Flashcards

1
Q

Which cytokine helps in terminal T and B cell production in BM?

A

IL-7

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2
Q

Which cytokine for megakaryocyte differentiation?

A

Thrombopoietin/IL-11

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3
Q

Which exon mutation in JAK2 in IE vs PV?

A

IE - exon 12 (only sometimes)

PV - exon 14

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4
Q

What can myeloproliferative disorders progress to?

A

AML or MF

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5
Q

Name 5 MPN.

A 
Polycythaemia vera (PV)
Essential Thrombocythaemia (ET)
Idiopathic Myelofibrosis
Idiopathic erythrocytosis
Chronic granulocytic leukaemia
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6
Q

What are the features of the following in terms of differentiation and proliferation levels:
Leukaemia
MPN
MDS

A

Leukaemia - no differentiation, just proliferation
MPN - proliferation and differentiation
MDS - no differentiation, no proliferation

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7
Q

What is low in PV?

A

EPO

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8
Q

What GI feature is common in PV?

A

Peptic ulceration

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9
Q

What is erythromelalgia?

A

Painful extremities

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10
Q

What is the BM like in PV?

A

Hypercellular - fat spaces occupied by erythroid cells

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11
Q

What test is used to rule out pseudopolycythaemia?

A

Isotope dilution test/Fick’s test
RBC with chromium
Plasma with iodine (binds albumin)

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12
Q

What are the causes of a raised haematocrit without JAK2 mutation?

A
  1. True polycythaemia (due to raised EPO e.g. hypoxia, renal tumour OR familial)
  2. Pseudopolycythaemia
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13
Q

What fibres are increased in BM in PV?

A

Reticulin fibres

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14
Q

What is the tx of IE?

A

Venesection only to reduce RBCs

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15
Q

What plt level suggests ET?

A

> 600x 10

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16
Q

What age does ET occur at?

A

Bimodal - 30 and 55

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17
Q

How does ET present?

A

Thrombosis or mucous bleeding

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18
Q

What does the BM look like in ET?

A

Normal or slightly hypercellular

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19
Q

What is the use of anagrelide in ET?

A

Antiplatelet - but not used much as accelerates myelofibrosis + palpitations/flushing,

No venesection used in ET.

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20
Q

Briefly what happens in IMF?

A

BM fibrosis and extramedullary haematopoiesis

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21
Q

Does IE progress to IMF?

A

No, only PV and ET

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22
Q

What serious liver-related complication may occur in IMF?

A

Budd-Chiari syndrome

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23
Q

What hypermetabolic features are seen in IMF?

A

Weight loss, fatigues, night sweats, hyperuricaemia

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24
Q

What are the two stages of IMF?

A

Prefibrotic - looks like ET

Fibrotic

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25
Q

What blood features are seen in IMF?

A

Tear drop poikilocytes
Leukoerythroblastic picture
Giant platelets
Megakaryocytes

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26
Q

What nucleated myeloid cells may be seen in the peripheries in IMF?

A

Basophils

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27
Q

Rank the MPNs in terms of best to worst prognosis.

A

IE - no adverse effects
ET - ~20yrs
PV - 10 yrs
IMF - 3-5yrs

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28
Q

What is the normal Hb and Hct in females?

A

115-165

0.37-0.47

29
Q

What is the normal Hb and Hct in males?

A

130-180

0.4-0.54

30
Q

What causes pseudopolycythaemia?

A

Alcohol, obesity, diuretics

31
Q

What are the causes of polycythaemia which are Ph+ve?

A

CML

32
Q

What are the Ph-ve causes of polycythaemia?

A

PV
ET
MF

33
Q

What haematopoietic precursor is taregted in PV?

A

BFU-E

34
Q

Which myeloid neoplasms do not cause polycythaemia?

A

AML, myelodysplasia

35
Q

What are the causes of polycythaemia with raised EPO?

A 
Appropriate:
COPD
CHD
High altitude 
High affinity Hb

Inappropriate:
Renal disease
Uterine myoma
Liver/lung tumours etc

36
Q

Compare the mutations present in PV, ET and IMF.

A

PV - JAK2
IMF - JAK2, calreticulin
ET - JAK2, Calreticulin, MPL

37
Q

What is inactive EPO usually bound to in the cell?

A

JAK2

38
Q

Does IE have the JAK2 mutation?

A

No

39
Q

Name 2 causes of massive hepatosplenomegaly.

A

CML and MF

40
Q

Which scoring system is used for prognosis in MF?

A

DIPPS - score of 6 means 1.3yr survival

41
Q

Which JAK2 inhibitor may sometimes be used in MF with poor prognosis?

A

Ruxolotinib

42
Q

How does CML differ from PMF/PV/ET?

A

It is PhChr +ve

43
Q

What is the mutation causing CML?

A

BCR-ABL Ph Chr mutation (t (9; 22) –> Ph Chr)

44
Q

What is the FBC in CML?

A

Hb/plt normal or raised BUT massive leucocytosis

45
Q

What white cells may be characteristically found in CML?

A

Basophils, myelocytes and neutrophils (all mature)

46
Q

Which drug has drastically changed the prognosis with CML?

A

Imatinib

47
Q

What was the clinical course of CML before imatinib?

A

Chronic phase

Advanced phase: (1) accelerated (10-19% blasts), (2) blast crisis (>20% blasts)

48
Q

What is the MOA of imatinib?

A

ABL TK inhibitor

49
Q

How do you monitor Imatinib therapy?

A

RQ-PCR of % BCR-ABL transcripts

50
Q

What is a major molecular response in CML to imatinib?

A

3 log reduction in BCR-ABL i.e. <0.1% AT 18 MONTHS

51
Q

What are 2 SE of TK inhibitors?

A

Fluid retention

Pleural effusion

52
Q

Name a 2nd and 3rd gen TK inhibitor.

A

1st Gen – Imatinib
2nd Gen – Dasatinib, Nilotinib
3rd Gen – Bosutinib

53
Q

What is myelodysplasia?

A

Abnormal myeloid clone forms + number is reduced

54
Q

What can myelodysplasia transform to?

A

AML

55
Q

What is Pelger-Huet abnormality?

A

Bilobed neutrophils - characteristically seen in myelodysplastic syndromes

56
Q

What is the name for an increased number of mature hypersegmented neutrophils seen in MDS?

A

Myelokathexis

57
Q

What stain is used to see ringed sideroblasts in MDS?

A

Prussian blue (haemosiderin inside the mitochondria of erythroid precursors forms a ring around the RBS nucleus)

58
Q

Where are Auer rods seen?

A

In myeloblasts

59
Q

What is the FBC in myelodysplasia?

A

Everything low

60
Q

What is the prognostic index in MDS?

A

IPSS - takes into account %BM blasts, karyotype, Hb, plt, neutrophils

61
Q

Which mutations confer good prognosis in MDS?

A

del(11q), (5q), (12q) and (20q)

62
Q

Which mutation confers a poor prognosis in MDS?

A

del(7q)

63
Q

What is the prognosis with AML from MDS?

A

Very poor and usually not curable

64
Q

What are the causes of death in MDS?

A

1/3 each from:
Infection
Bleeding
AML

65
Q

Which MDS treatments prolong survival?

A

Allo SCT

Intensive chemo

66
Q

What is the MOA of -CYTIDINES in MDS?

A

hypomethylating agents

67
Q

When is lenalidomide used in MDS?

A

In those with del(5q) (generally this confers a good prognosis)

68
Q

Which low-dose PO and SC chemo may be used in MDS?

A

Hydroxyurea(PO) and cytarabine (SC)

Pathology Summaries (23 decks)

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