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Haematology > Myeloproliferative Neoplasms > Flashcards

Myeloproliferative Neoplasms Flashcards

1
Q

myeloproliferative neoplasms (MPN) used to be called what?

A

myeloproliferative disorders

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2
Q

what does myeloproliferative mean?

A

proliferation of bone marrow/bone marrow lineages

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3
Q

myeloproliferative neoplasms are clonal haematopoietic stem cell disorders, what does this mean?

A

increased production of one or more types of haematopoietic cells
maturation is relatively preserved (in contract to acute leukaemia)

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4
Q

sub-types of myeloproliferative neoplasms?

A

BCR-ABL1 negative

BCR-ABL1 positive

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5
Q

types of BCR-ABL1 negative myeloproliferative neoplasms?

A 
primary myelofibrosis
polycthaemia vera (overproduction of RBCs)
essential thrombocytopenia (overproduction of platelets)
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6
Q

types of BCR-ABL1 positive myeloproliferative disorders

A

chronic myeloid leukaemia (overproduction of granulocytes, Philadelphia chromosome)

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7
Q

when should a myeloproliferative neoplasm be considered as a diagnosis?

A 
high granulocyte count 
\+/-
high RBCs/Hb
\+/-
high platelets
\+/- 
eosinophilia/basophila
splenomegaly
thrombosis in unusual place
(must have no reactive explanation)
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8
Q

what happens in CML?

A

proliferation of myeloid lineage cells (granulocytes and their precursors, other lineages including platelets)
previously, had a chronic phase with intact maturation of cells for 3-5 years followed by “blast crisis” reminiscent of acute leukaemia with maturation defect

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9
Q

CML is fatal in the chronic phase without what?

A

stem cell/bone marrow transplant

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10
Q

3 phases of CML?

A

chronic phase
accelerated phase
blast crisis

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11
Q

clinical features of CML?

A 
asymptomatic
splenomegaly
hypermetabolic symptoms
gout
miscellaneous (problems related to hyperleukocytosis problems, priapism)
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12
Q

lab features of CML?

A

blood count changes

- normal/reduced Hb, leucocytosis with neutrophilia and myeloid precursors, eosinophilia, basophilia, thrombocytosis

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13
Q

what is the hallmark of CML?

A

Philadelphia chromosome

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14
Q

genetics of CML?

A

Philadelphia chromosome results in a new chimaeric gene (BCR-ABL1)
the gene produces a tyrosine kinase which causes abnormal phosphorylation (signalling) leading to haematological changes in CML

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15
Q

types of BCR-ABL1 negative myeloproliferative neoplasms

A 
polycthaemia vera (PV)
essential thrombocytopenia (ET)
primary myelofibrosis (PMF)
others
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16
Q

features common to myeloproliferative neoplasms?

A 
asymptomatic
increased cell turnover (gout, fatigue, weight loss, sweats)
signs of splenomegaly
marrow failure
thrombosis (arterial and venous)
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17
Q

what is PV?

A

polycthaemia vera
high Hb/haematocrit accompanied by erythrocytosis (true increase in RBC mass) but can have excessive production of other lineages

18
Q

how can PV be distinguished from secondary polycthaemia?

A

secondary associated with chronic hypoxia, smoking and erythropoietin secreting tumour etc

19
Q

how can PV be distinguished from pseudopolycthaemia?

A

pseudopolycthaemia associated with dehydration, diuretic therapy, obesity etc

20
Q

true vs pseudo polycthaemia?

A 
true = actual increase in number of RBCs
pseudo = same amount of RBCs but RBCs are dissolved in less plasma so looks like relatively more RBCs
21
Q

clinical features of PV?

A

same features common to all MPNs
headache
fatigue (result of increased BLOOD viscosity not plasma)
itch (aguagenic pruritis)

22
Q

how is polycthaemia investigated?

A

history
examination (splenomegaly etc)
FBC
blood film
JAK2 mutation status (IMPORTANT)
investigate for secondary/pseudo causes (CXR, SpO2, ABGs)
other tests (erythropoietin, bone marrow biopsy done rarely)

23
Q

what is JAK2 mutation?

A

JAK2 = a kinase
JAK2 mutation results in loss of auto-inhibition and activation of erythropoiesis present
present in 95% of PV cases, therefore part of screening for PV

24
Q

how is PV managed?

A

venesect until haematocrit <0.45
aspirin
cytotoxic oral chemotherapy (hydroxycarbamide)

25
Q

what is ET?

A

essential thrombocytopenia
uncontrolled production of abnormal platelets resulting in thrombosis and bleeding at high levels due to von Willebrand disease

26
Q

clinical features of ET?

A

features common to all MPNs (particularly vaso-occlusive complications)
bleeding

27
Q

how is ET diagnosed?

A

exclude reactive thrombocytosis (IMPORTANT)
exclude CML
check for genetic mutations (JAK2, CALR, MPL, some can be triple negative)
characteristic bone marrow appearance

28
Q

how is ET managed?

A

anti-platelets (aspirin)

cytoreductive therapy to control proliferation (hydroxycarbamide, anagrelide, interferon alpha)

29
Q

what can cause myelofibrosis?

A

idiopathic
post-polycthaemia
essential thrombocytopenia

30
Q

features of idiopathic myelofibrosis?

A

marrow failure
bone marrow fibrosis with no alternative cause
extramedullary haematopoiesis (in liver and spleen)

31
Q

blood film appearance of idiopathic myelofibrosis (important)

A

leucoerythroblastic film appearance

teardrop-shaped RBCs in peripheral blood

32
Q

clinical features of myelofibrosis?

A

marrow failure (anaemia, bleeding, infection)
spelomegaly (LUQ pain, complications including portal hypertension)
hypercatabolism
also the clinical features common to all MPNs

33
Q

lab diagnosis of myelofibrosis?

A

typical blood film (teardrop RBCs - “poikilocytes” and leucoerythroblastic)
dry aspirate
fibrosis on trephine biopsy (bone section)
JAK2, CALR, MPL mutations

34
Q

causes of leucoerythroblastic film?

A

sepsis (reactive bone marrow)
marrow infiltration
myelofibrosis

35
Q

what is leucoerythroblastic film?

A

presence of immature RBCs and WBCs

36
Q

how is myelofibrosis managed?

A

supportive care (blood transfusions, antibiotics)
allogenic stem cell transplant in some
splenectomy (controversial)
JAK2 inhibitors

37
Q

most common cause of high cell counts?

A

most commonly reactive

38
Q

reactive causes of high granulocytes?

A

infection

physiological (post surgery, steroids etc)

39
Q

reactive causes of high platelets?

A

infection
iron deficiency
malignancy
blood loss

40
Q

reactive causes of high RBCs?

A

dehydration (diuretics) i.e - pseudopolycthaemia

secondary polycthaemia

Haematology (26 decks)

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