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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

Is maturation preserved in myeloproliferative neoplasms?

A

Yes = it is relatively preserved

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3
Q

What does microscopy of myeloproliferative neoplasm show?

A

Hypercellular with a variety of cells at different stages of development

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4
Q

How are myeloproliferative neoplasms classified?

A

Broadly split using BCR-ABL1 = can be positive or negative

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5
Q

What is an example of a BCR-ABL1 positive myeloproliferative neoplasm?

A

Chronic myeloid leukaemia = overproduction of granulocytes

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6
Q

What are some examples of BCR-ABL1 negative myeloproliferative neoplasms?

A

Primary myelofibrosis
Polycythaemia vera = red cell overproduction
Essential thrombocythaemia = platelet overproduction

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7
Q

When would you consider myeloproliferative neoplasm as a diagnosis?

A

High granulocyte count +/- high red cell count +/- high platelet count +/- eosinophilia/basophilia +/- splenomegaly and thrombosis in unusual places

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8
Q

What must be excluded before diagnosing myeloproliferative neoplasm?

A

Reactive causes of blood abnormalities

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9
Q

What is chronic myeloid leukaemia?

A

Proliferation of myeloid cells = granulocytes and their precursors, platelets

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10
Q

What is the course of chronic myeloid leukaemia?

A

Chronic phase = intact maturation, 3-5 years

Blast crisis = lack of progenitors (view on blood film)

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11
Q

What is the prognosis of chronic myeloid leukaemia?

A

Fatal without stem cell or bone marrow transplant in chronic stage

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12
Q

What are the features of chronic myeloid leukaemia?

A

Asymptomatic, splenomegaly, hypermetabolic syndrome, gout, priaprism, problems related to hyperleucocytosis

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13
Q

What lab results would you expect in a patient with chronic myeloid leukaemia?

A

Normal or reduced Hb, leucocytosis with neutrophilia and myelocytes, eosinophilia, basophilia, thrombocytosis

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14
Q

What does bone marrow biopsy of chronic myeloid leukaemia show?

A

Hypercellularity

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15
Q

What is the hallmark of chronic myeloid leukaemia?

A

Philadelphia chromosome = due to transolcation between chromosomes 9 and 22

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16
Q

What does the Philadelphia chromosome cause?

A

Chimeric BCR-ABL1 gene = tyrosine kinase is produced which leads to haematological changes due to abnormal phosphorylation

17
Q

What treatment targets the Philadelphia chromosome to treat chronic myeloid leukaemia?

A

Tyrosine kinase inhibitors = imatinib

18
Q

What patient age group is most affected by BCR-ABL1 negative myeloproliferative neoplasms?

A

Patients aged >65

19
Q

What are the common features of the BCR-ABL1 negative myeloproliferative neoplasms?

A

Asymptomatic, fatigue, weight loss, sweats, gout, splenomegaly, marrow failure (esp primary myelofibrosis), thrombosis, erythromelalgia

20
Q

What are the characteristics of polycythaemia vera?

A

High Hb and haematocrit accompanied by erythrocytosis = causes true increase in red cell mass

21
Q

How do you distinguish between secondary polycythaemia and pseudo-polycythaemia?

A

Using a centrifuge = shows increase in red cell mass present in polycythaemia

22
Q

What are the features of polycythaemia vera?

A

Headache, fatigue, itch (exacerbated by warm water)

23
Q

What investigations are done for polycythaemia vera?

A

FBC, blood film, JAK2 mutation analysis (present in 95%)

24
Q

What is the JAK2 mutation?

A

Mutation in kinase = results in loss of auto-inhibition and activation or erythropoiesis in absence of ligand

25
Q

How is polycythaemia vera treated?

A

Venesect to haematocrit <0.45, aspirin, cytotoxic oral chemotherapy (e.g hydroxycarbamide)

26
Q

What occurs in essential thrombocythaemia?

A 
Uncontrolled production of abnormal platelets 
Abnormal platelet function = thrombosis, vWF disease at high level (>1500) causing bleeding
27
Q

What are the features of essential thrombocythaemia?

A

Veno-occlusive features, bleeding

28
Q

What must be excluded before essential thrombocythaemia can be diagnosed?

A

Reactive causes and chronic myeloid leukaemia

29
Q

What are the genetics associated with essential thrombocythaemia?

A

50-60% have JAK2 mutation, 25% have CALR mutation and 5% have MPL mutation
10-20% have none of these mutations

30
Q

What is the main diagnostic test for essential polycythaemia?

A

Bone marrow biopsy

31
Q

What is the treatment of essential polycythaemia?

A

Aspirin

Cytoreductive therapy = hydroxycarbamide mainly, also angrelide or INF-alpha

32
Q

What is the least common BCR-ABL1 negative myeloproliferative neoplasm?

A

Primary myelofibrosis = can be idiopathic or post-polycythaemia/essential thrombocythaemia

33
Q

What are the features of primary myelofibrosis?

A

Marrow failure, bone marrow fibrosis, extramedullary haemopoiesis (hepatosplenomegaly), anaemia, bleeding, infections, LUQ pain, hypercatabolism (e.g weight loss)

34
Q

What is the blood film appearance of primary myelofibrosis?

A

Leukoerythroblastic blood film with teardrop shaped red cells in the peripheral blood

35
Q

How is primary myelofibrosis diagnosed?

A

Blood film = characteristic appearance
Bone marrow aspirate = expect dry tap
Trephine biopsy = shows fibrosis

36
Q

What are the genetics associated with primary myelofibrosis?

A

Most patients are JAK2 positive, followed by CALR then MPL mutations
10% have none of these mutations

37
Q

What is a leucoerythroblastic film appearance?

A

Presence of both red cell and neutrophil precursors in blood = can also be caused by sepsis and marrow infiltration

38
Q

What is the treatment of primary myelofibrosis?

A

Supportive = blood transfusion, platelets, antibiotics
Allogenic stem cell transplant in select few
JAK2 inhibitors = improve spleen size and symptoms
Splenectomy = less common and controversial

39
Q

Are myeloproliferative neoplasms a common cause of abnormally high cell counts?

A

No = reactive causes of raised counts are more common

Haematology (24 decks)

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