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Year 3 - Haematology > Myeloproliferative neoplasms > Flashcards

Myeloproliferative neoplasms Flashcards

1
Q

What is the new term for myeloproliferative disorders?

A

Myeloproliferative neoplasms

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2
Q

What is the new term for polycythemia rubra vera?

A

Polycythemia vera

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3
Q

What does myeloproliferative mean?

A

Bone marrow lineages (granulocytes, red cells, platelets)

To grow or multiply by rapidly producing new tissue

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4
Q

What are myeloproliferative neoplasms?

A

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

In contrast to acute leukemia, maturation is relatively preserved

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5
Q

What is the BCR-ABL1 positive myeloproliferative neoplasm?

A

Chronic myeloid leukemia (over production of granulocytes)

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6
Q

What chromosome is associated with chronic myeloid leukemia?

A

Philadelphia chromosome

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7
Q

What myeloproliferative neoplasms are BCR-ABL1 negative?

A

Primary myelofibrosis
Polycythaemia vera (over production of red cells)
Essential thrombocythaemia (overproduction of platelets)

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8
Q

When should you consider a myeloproliferative neoplasm?

A

High granulocyte count
+/-
High red cell count (haemoglobin)
+/-
high platelet count
+/-
eosinophilia/basophilia

Splenomegaly
Thrombosis in an unusual place

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9
Q

What is chronic myeloid leukemia?

A

Proliferation of myeloid cells
- Granulocytes and their precursors
- Other lineages (platelets)

Chronic phase with intact maturation 3-5 years, followed by blast crisis reminiscent of acute leukemia with maturation defect

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10
Q

What are the phases of chronic myeloid leukemia

A
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11
Q

What are symptoms and signs of chronic myeloid leukemia?

A

Asymptomatic
Splenomegaly
Hypermetabolic symptoms
Gout
Miscellaneous: problems related to hyperleucocytosis problems, priapism

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12
Q

What are lab findings in chronic myeloid leukemia?

A

Normal or low Hb
Leucocytosis with neutrophilic and myeloid precursors (myelocytes), eosinophilia, basophilia
Thrombocytosis

Bone marrow

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13
Q

Which one is myeloproliferative neoplasm (CML) vs reactive?

A

Left: MPN
Right: Reactive

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14
Q

What is the hallmark finding in chronic myeloid leukemia?

A

Philadelphia chromosome

Translocation of chromosomes 9 and 22 (BCR-ABL)

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15
Q

Describe what genetic changes occur to cause chronic myeloid leukemia

A

Philadelphia chromosome results in a new chimeric gene (BCR-ABL1)

The gene product is a tyrosine kinase which causes abnormal phosphorylation (signalling) leading to the haematological changes in CML

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16
Q

What medication can be used to treat chronic myeloid leukemia?

A

Tyrosine kinase inhibitors e.g. imatinib

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17
Q

What are features common to myeloproliferative neoplasms?

A

Asymptomatic
Increased cellular turnover (gout, fatigue, weight loss, sweats)
Symptoms / signs due to splenomegaly
Marrow failure (fibrosis or leukemic transformation: lower with PRV and ET)
Thrombosis (arterial or venous including TIA, MI, abdominal vessel thrombosis, claudication, erythromelalgia)

18
Q

What is polycythemia vera?

A

High Hb / Haematocrit accompanied by erythrocytosis (a true increase in red cell mass) but can have excessive production of other lineages

19
Q

What are causes of secondary polycythemia?

A

Chronic hypoxia
Smoking
Erythropoeitin secreting tumour

20
Q

What are causes of pseudopolycythemia?

A

Dehydration
Diuretic therapy
Obesity

21
Q

What would true polycythemia vs pseudopolycythemia present after centrifuging blood?

A
22
Q

What are symptoms & signs of polycythemia vera?

A

Clinical features common to MPN
Headache, fatigue (remember blood viscosity raised not plasma viscosity)
Itch (Aquagenic puritis)

23
Q

What examinations do we do for polycythemia vera?

A

History - e.g. history suggestive of secondary polycythemia?
Examination - e.g. splenomegaly
FBC, film
JAK2 mutation status

24
Q

What is JAK2?

A

A kinase

25
Q

What mutation is seen in PRV?

A

JAK2 mutations present in 95% of these patienst
Mutation (substitution) results in loss of auto-inhibition
Activation of erythropoeisis in absence of ligand

Mutational analysis forms part of initial screening

26
Q

How can we investigate for polycythemia?

A

Investigation for secondary / pseudo causes (CXR, O2 saturation / ABGs, drug history)

JAK2 analysis
Infrequent tests: erythropoeitin levels, bone marrow biopsy

27
Q

How do you treat polycythemia vera?

A

Venesection (until haematocrit <0.45)

Aspirin

Cytotoxic oral chemotherapy (e.g. hydroxycarbamide)

28
Q

What is essential thrombocythemia?

A

Uncontrolled production of abnormal platelets

Platelet function abnormal - thrombosis; at high levels can also cause bleeding due to acquired von willebrand disease

29
Q

What are clinical features of essential thrombocythemia?

A

Vasoocclusive complications
Clinical features common to MPN
Bleeding

30
Q

How do we diagnose essential thrombocythemia?

A

Exclude reactive thrombocytosis (blood loss, inflammation, malignancy, iron deficiency)
Exclude CML
Genetics: JAK2 mutations in 50-60%; CALR (calreticulin) in approx 25%; MPL mutation in 5%; 10-20% of patients will be triple negative

31
Q

What treatment do we give for essential thrombocythemia?

A

Anti-platelet agents: aspirin
Cytoreductive therapy to control proliferation: hydroxycarbamide, anagrelide, interferon alpha

32
Q

What are causes of myelofibrosis?

A

Idiopathic
Post-polycythemia
Essential thrombocythemia

33
Q

What is idiopathic myelofibrosis signs and clinical features?

A

Marrow failure
Bone marrow fibrosis
Extramedullary haumatopoeisis (liver and spleen)
Leukoerythroblastic film appearances
Teardrop shaped RBCs in peripheral blood

34
Q

What are signs of myelofibrosis?

A

Marrow failure - bleeding, anaemia, infection
Splenomegaly - LUQ abdominal pain, complications including portal hypertension
Hypercatabolism

35
Q

How do we diagnose myelofibrosis?

A

Typical blood film (tear drop shaped RBC and leukoerythroblastic)
Dry aspirate
Fibrosis on trephine biopsy
JAK2, CALR, MPH mutations

36
Q

What are causes of leukoerythroblastic film?

A
37
Q

What is treatment for myelofibrosis?

A

Supportive care (blood transfusion, platelets, antibiotics)
Allogenic stem cell transplantation in few
Splenectomy (controversial)
JAK2 inhibitors

38
Q

What are reactive causes of high counts of granulocytes?

A

Infection - e.g. pyogenic bacteria causing neutrophilia
Physiological - e.g. post-surgery, steroids

39
Q

What are reactive causes of high counts of platelets?

A

Infection
Iron deficiency
Malignancy
Blood loss

40
Q

What are reactive causes of high counts of red cells?

A

Dehydration (diuretics) - pseudopolycythemia
Secondary polycythemia e.g. hypoxia induced

Year 3 - Haematology (35 decks)

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