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Haematology > Myeloproliferative Neoplasms > Flashcards

Myeloproliferative Neoplasms Flashcards

1
Q

what are myeloproliferative neoplasms

A

Clonal haemopoietic stem cell disorders with an increased production of one or more types of haemopoietic cells

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2
Q

chronic myeloid leukaemia has mostly overproduction of _______

A

granulocytes

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3
Q

polycythaemia - overproduction of ______

A

RBCs

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4
Q

when to consider an MPN

A

High Granulocyte count
and/or
High Red cell count / haemoglobin
and/or
High Platelet count
and/or
Eosinophilia/basophilia

Splenomegaly

Thrombosis in an unusual place

AND NO REACTIVE EXPLANATION

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5
Q

which chronic condition leads to chronic hypoxia and Secondary erythrocytosis

A

COPD

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6
Q

chronic myeloid leukaemia phases

A
  • chronic phase (3-5 years?)
  • accelerated phase (transition to maturation defect)
  • blast crisis
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7
Q

CML clinical features

A

Asymptomatic

Splenomegaly

Hypermetabolic symptoms

Gout

Misc: Problems related to hyperleucocytosis problems, Priapism

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8
Q

CML laboratory features - blood count changes

A

normal/↓Hb
leucocytosis with neutrophilia and myeloid precursors (myelocytes), eosinophilia, basophilia
thrombocytosis

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9
Q

___________ chromosome is the hallmark of CML

A

philadelphia

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10
Q

CML - Philadelphia chromosome results in a new gene: ________

A

BCR-ABL-1

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11
Q

CML treatment

A

Fatal without stem cell/bone marrow transplant in the chronic phase

double treatment with a type of targeted cancer drug called a tyrosine kinase inhibitor (TKI) - e.g. imatinib

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12
Q

features common to myeloproliferative neoplasms

A

Asymptomatic

Increased cellular turnover (gout, fatigue, weight loss, sweats)

Symptoms/signs due to splenomegaly

Marrow failure (fibrosis or leukaemic transformation:lower with PRV and ET)

Thrombosis (arterial or venous including TIA, MI, abdominal vessel thrombosis, claudication, erythromelalgia)

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13
Q

what is polycythaemia vera (PV)?

A

High haemoglobin/haematocrit accompanied by erythrocytosis (a true increase in red cell mass) but can have excessive production of other lineages (white cells, platelets)

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14
Q

what is it important to distinguish polycythaemia vera from?

A

secondary polycythaemia (chronic hypoxia, smoking, erythropoietin-secreting tumour etc)

pseudopolycythaemia (eg dehydration, diuretic therapy, obesity)

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15
Q

what causes haematocrit to be raised in pseudopolycythaemia?

A

decrease in plasma volume
e.g. dehydration, diuretic therapy, obesity

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16
Q

polycythaemia vera clinical features

A

Clinical features common to MPN

Headache, fatigue (remember blood viscosity raised NOT plasma viscosity)

Itch (aquagenic puritis)

17
Q

investigation of polycythaemia (clinical and lab)

A

History (eg history suggestive of a secondary polycythaemia?)

Examination (eg splenomegaly?)

FBC, film

JAK2 mutation status (IMPORTANT)

–
Investigation for secondary/pseudo causes (CXR, O2 saturation/arterial blood gases, drug history)

JAK2 analysis

18
Q

what percentage of patients with polycythaemia vera have JAK2 mutation

A

95%

19
Q

Polycythaemia vera treatment

A
  • Vensect to haematocrit <0.45
  • Aspirin
  • Cytotoxic oral chemotherapy (eg Hydroxycarbamide)
20
Q

what is Essential Thrombocythaemia (ET)

A

Uncontrolled production of abnormal platelets

21
Q

Essential Thrombocythaemia.
platelet function abnormal - what can this cause?

A
  • thrombosis
  • at high levels can also cause bleeding due to acquired von Willebrand disease
22
Q

Essential Thrombocythaemia clinical features

A

Clinical features common to MPN (particularly vasoocclusive complications)

Bleeding (unpredictable risk especially at surgery)

23
Q

if suspecting essential thrombocythaemia, what is it important to exclude?

A

reactive thrombocytosis (Blood loss, inflammation, malignancy, iron deficiency)

also exclude CML

24
Q

Essential Thrombocythaemia genetics and percentages of patients positive for these mutations

A

JAK2 mutations in approx. 50 - 60%
CALR (Calreticulin) in approx. 25%
MPL mutation in approx. 5%

10-20% of patients will be ‘Triple Negative’

25
Q

Essential Thrombocythaemia treatment

A

Anti-platelet agents
- Aspirin

Cytoreductive therapy to control proliferation
- hydroxycarbamide, anagrelide, interferon
alpha

26
Q

Myelofibrosis presentation (on testing)

A

Marrow failure (variable degrees)
Bone marrow fibrosis (no alternative cause)
Extramedullary hematopoiesis (liver and spleen)
Leukoerythroblastic film appearances
- IMPORTANT!!
Teardrop-shaped RBCs in peripheral blood

27
Q

myelofibrosis clinical features

A

Clinical features common to MPD

Marrow failure
- anaemia, bleeding, infection

Splenomegaly
- LUQ abdominal pain
- Complications including portal
hypertension

Hypercatabolism

28
Q

myelofibrosis lab diagnosis

A

typical blood film (tear-drop shaped RBC and leucoerythroblastic)

dry aspirate

fibrosis on trephine biopsy

JAK2, CALR, MPL mutations (majority JAK2 > CALR > MPL. Approx 10% ‘triple negative’)

29
Q

why dry tap/aspirate in lab diagnosis of myelofibrosis

A

scar tissue

30
Q

leucoerythroblastic film is characterised by…

A

characterized by the presence of immature forms of red and white cells in the peripheral blood

31
Q

causes of leucoerythroblastic blood film

A
  • reactive (sepsis)
  • marrow infiltration
  • myelofibrosis
32
Q

myelofibrosis treatment

A

Supportive care (blood transfusion, platelets, antibiotics)

Allogeneic stem cell transplantation in a select few (if young and fit, high risk disease)

Splenectomy (CONTROVERSIAL, rarely performed)

JAK2 inhibitors (improve spleen size, constitutional symptoms, ?survival)

33
Q

in high blood counts, which is more common, myeloproliferative neoplasms or reactive causes

A

reactive causes

34
Q

causes of raised platelet count apart from myeloproliferative neoplasms

A

Infection
Iron deficiency
Malignancy
Blood loss

35
Q

causes of raised granulocyte count apart from myeloproliferative neoplasms

A

Infection: eg pyogenic bacteria causing neutrophilia

Physiological eg post-surgery, steroids

36
Q

causes of raised red cell count apart from myeloproliferative neoplasms

A

Dehydration (diuretics): pseudopolycythaemia

Secondary polycythaemia (eg hypoxia-induced)

Haematology (36 decks)

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