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Haematology > Chronic Myeloproliferative Disorders > Flashcards

Chronic Myeloproliferative Disorders Flashcards

1
Q

What does proliferative mean?

A

To grow or multiple by rapidly producing new tissue, parts, cells or offspring

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2
Q

Describe myeloproliferative disorders

A

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

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3
Q

Is maturation depleted or preserved?

A

Preserved

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4
Q

What is BCR-ABL1 +ve?

A

Chronic myeloid leukaemia

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5
Q

What is BCR-ABL -ve?

A

Idiopathic myelofibrosis
Polycythaemia rubra vera
Essential thrombocytopenia

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6
Q

When would you consider a myeloproliferative disorder?

A 
High granulocyte count
High red cell count/Hb
High platelet count
Eosinophilia/basophilia
Splenomegaly
Thrombosis in an unusual place
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7
Q

What is Chronic Myeloid Leukaemia? (CML)

A

Proliferation of myeloid cells

Granulocytes and their precursors and platelets

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8
Q

What is needed to help prevent CML from being fatal?

A

Stem cell / bone marrow transplantation in the chronic phase

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9
Q

What are the 3 phases of CML?

A

Chronic
Accelerated
Blast crisis

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10
Q

What are clinical features of CML?

A 
Asymptomatic
Splenomegaly
Hypermetabolic symptoms
Gout
Problems related to hyperleucocytosis and priapism
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11
Q

What are lab features of CML?

A

Normal or reduced Hb
Leukocytosis with neutrophilia and myeloid precursors (myelocytes), eosinophilia, basophilia
thrombocytosis
Bone marow changes

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12
Q

What is the chromosome, the gene and the product which causes the abnormal phosphorylation (signalling) leading to the haematological changes seen in CML?

A 
Chromosome = Philadelphia chomosome (22)
Gene = BCR-ABL1
Product = Tyrosine kinase
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13
Q

What can be used to treat CML?

A

Imatinib

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14
Q

Describe Polycthaemia rubra vera? (PRV)

A

High haemoglobin / haematocrit accompanied by arythrocytosis (a true increase in red cell mass) but can have excessive production of other lineages

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15
Q

Describe secondary polycythaemia?

A

Chronic hypoxia
Smoking
Erythropoietin-secreting tumour

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16
Q

Describe pseudopolycthaemia?

A

Dehydration
Diuretic therapy
Obesity

17
Q

What are clinical features of PRV?

A

Clinical features common to MPD?
Headache
Fatigue
Itch (aquagenic puritis)

18
Q

What happens with the Philadelphia chromosome?

A

22 and 9 swap some bits of information

Translocation

19
Q

How do you investigate polycthaemia?

A

History
Examination
FBC + film
JAK2 mutation status

20
Q

Describe JAK2?

A

It’s a kinase
Mutation present in over 95% of PRV patients
Substitution results in loss of auto-inhibition
Activation of erythropoiesis in the absence of ligand

21
Q

What are some infrequent tests for polycythaemia?

A

Erythropoietin levels

Bone marrow biopsy

22
Q

What are investigations for secondary polycythaemia?

A

CXR
O2 saturations
Arterial blood gas
Drug history

23
Q

What is the treatment for PRV?

A

Vensect to haemocrit <0.45
Aspirin
Cytotoxic oral chemo (e.g. hydroxycarbamide)

24
Q

Describe ET

A

Uncontrollable production of abnormal platelets with abnormal function
Thrombosis
Can cause bleeding due to acquired von Willebrand disease

25
Q

What are clinical features of ET?

A

Common to MPD (esp. vasooclusive complications

Bleeding

26
Q

How do you diagnose ET?

A 
Exclude reactive thrombocytosis
Exclude CML
Genetics: JAK2 (50%)
CALR (calreticulin)
MPL
Bone marrow appearances
27
Q

How do you treat ET?

A

Anti-platelet agents (aspirin)

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

28
Q

What can myelofibrosis be?

A

Idiopathic
Agnogenic myeloid metaplasia
Post-polycythaemia or essential thrombocythaemia

29
Q

Describe idiopathic myelofibrosis?

A 
Marrow failure
Bone marrow fibrosis
Extramedullary hematopoiesis
Leukoerythroblastic 
Teardrop-shaped RBC's in peripheral blood
30
Q

What are clinical features of myelofibrosis?

A

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

31
Q

How do you diagnose myelofibrosis?

A

Typical blood film (tear-drop shaped RBC and leucoerythroblastic)
Fibrosis on trephine biopsy
JAK2 or CALR mutation

32
Q

What are a leucoerythroblastic film ?

A

Reactive (sepsis)
Marrow infiltration
Myelofibrosis

33
Q

How do you treat myelofibrosis?

A

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

34
Q

What counts as abnormal platelets?

A

Infection increase
Iron deficiency
Malignancy
Blood loss

Haematology (27 decks)

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