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y3 haematology > Myeloproliferative Disorders > Flashcards

Myeloproliferative Disorders Flashcards

1
Q

What are myeloproliferative disorders (MPD)?

A

Haemopoietic stem cell disorders involving increased production with preservation of maturation

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2
Q

How are myeloproliferative disorders (MPD) different from acute leukaemia?

A

Maturation is relatively preserved

Mature cells seen on film, whereas in leukaemia they are monomorphic nucleated cells (‘blasts)

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3
Q

What are the 2 groups/sub-types of myeloproliferative disorders?

A

BCR-ABL1 negative

BCR-ABL2 positive

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4
Q

Which MPD is BCR-ABL2 positive?

A

Chronic myeloid leukaemia

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5
Q

Which MPDs are BCR-ABL2 negative?

A

Essential thrombocythaemia
Polycythaemia rubra vera
Idiopathic myelofibrosis

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6
Q

When should an MPD be considered?

A

High granulocyte count (chronic myeloid leukaemia)
High red cell count (polycythemia rubra vera)
High platelet count (thrombocythaemia)
Eosinophilia/basophilia
Splenomegaly
Thrombosis in an unusual place

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7
Q

What happens in chronic myeloid leukaemia?

A

Excess proliferation of myeloid cells (granulocytes, platelets)
3-5 years chronic phase followed by “blast” crisis akin to acute leukaemia which can be fatal

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8
Q

What would the blood film of someone with chronic phase CML look like?

A

Lots of mature neutrophils

Increased primitive cells

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9
Q

What would the blood film of someone with accelerated phase CML look like?

A

Some neutrophils

Increased primitive cells

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10
Q

What would the blood film of someone with blast crisis in CML look like?

A

Monomorphic nucleated blasts (myelocytes)

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11
Q

List some clinical features of chronic myeloid leukaemia

A 
Asymptomatic
Splenomegaly (fatigue, LUQ pain)
Hypermetabolic symptoms (sweats, fever)
Weight loss
Bone pain
Gout
Miscellaneous (priapism, visual blurring)
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12
Q

Describe the bone marrow of a patient with CML

A

Increased myelocytes and eosinophils

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13
Q

Hb may be normal in chronic myeloid leukaemia. True/False?

A

True

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14
Q

Which chromosomes are affected in chronic myeloid leukaemia? What is the genetic change and resultant protein?

A

“Philadelphia” chromosomes
9 and 22 translocation
Produces tyrosine kinase which causes abnormal phosphorylation and production of BCR-ABL1

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15
Q

List some clinical features of BCR-ABL1 negative myeloproliferative disorders

A

Asymptomatic
Increased cellular turnover (gout, fatigue, weight loss, sweats)
Signs due to splenomegaly
Marrow failure (fibrosis or leukaemic transformation)
Thrombosis (arterial or venous) e.g. TIA, MI

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16
Q

What is erythromelaegia?

A

It is characterized by intense, burning pain of affected extremities, severe redness (erythema), and is associated with myeloproliferative disorders

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17
Q

What is polycythaemia rubra vera?

A

High haemoglobin/haematocrit accompanied by erythrocytosis (overproduction of red cells) but can have excessive production of other lineages

18
Q

How is polycythaemia rubra vera distinguished from secondary polycythaemia?

A

Secondary polycythaemia is caused by smoking, chronic hypoxia, erythropoietin-secreting tumours

19
Q

How is polycythaemia rubra vera distinguished from pseudo-polycythaemia?

A

Pseudo-polycythaemia is caused by dehydration, diuretic therapy, obesity
i.e. plasma volume has shrunk which makes it seem like Hb is high but it is not

20
Q

List some clinical features of polycythaemia rubra vera

A

Headache
Fatigue
Itch (aquagenic puritis)
MPD features

21
Q

What is aquagenic puritis?

A

Itch worse on exposure to warm water

22
Q

List investigations for polycythaemia

A

History (triggers of secondary polycythaemia)
Examination e.g. splenomegaly
FBC, film
Investigation for secondary/pseudo causes (CXR, O2 sats/ABG, drug history)
Bone marrow biopsy

23
Q

Splenomegaly will not be present in secondary polycythaemia. True/False?

A

True

24
Q

Which gene mutation is important to investigate as a diagnostic tool in polycythaemia?

A

JAK2 - a kinase mutation present in 95% patients

Activation of erythropoeisis in absence of ligand

25
Q

How is polycythaemia rubra vera treated?

A

Venesection until haematocrit less than 0.45
Aspirin
Cytotoxic oral chemotherapy e.g. hydroxycarbamide

26
Q

What is essential thrombocythaemia?

A

Uncontrolled production of abnormal platelets, resulting in abnormal platelet function

27
Q

What other clinical features, in addition to those common to MPD is present in patients with essential thrombocythaemia?

A

Thrombosis (abnormal platelet function)

Bleeding (unpredictable risk especially at surgery)

28
Q

What gene mutations can be present in essential thrombocythaemia?

A

JAK2
CALR
MPL

29
Q

What is the characteristic bone marrow appearance of a patient with essential thrombocythaemia?

A

Lots of big platelets

Clustering of megakaryocytes

30
Q

How is essential thrombocythaemia treated?

A

Aspirin

Cytoreductive therapy to control proliferation (hydroxycarbamide, anagrelide, IFNalpha)

31
Q

What is idiopathic myelofibrosis?

A

Proliferation of an abnormal clone of hematopoietic stem cells in the bone marrow and other sites results in fibrosis of the bone marrow

32
Q

List clinical features of myelofibrosis, other than those common to MPD

A 
Marrow failure (anaemia, bleeding, infection)
Extramedullary haematopoeisis (splenomegaly)
Hypercatabolism (weight loss, cachexia)
33
Q

Describe the appearance of a blood film in myelofibrosis

A

Leucoerythroblastic
Teardrop-shaped RBC’s in peripheral blood
(RBCs squeezing out of a fibrotic bone marrow)

34
Q

What is meant by a leucoerythroblastic film?

A

Erythroblast, red cell precursor, and myelocyte, granulocyte precursors in peripheral blood

35
Q

List causes of a leucoerythroblastic film

A

Reactive (sepsis)
Marrow infiltration
Myelofibrosis

36
Q

List diagnostic tests for myelofibrosis

A

Blood film
Dry aspirate
Fibrosis on trephine biopsy
JAK2 or CALR mutation in a proportion

37
Q

List treatment options for myelofibrosis

A

Supportive care (blood transfusion, platelets, abx)
Allogenic stem cell transplantation in young
Splenectomy
JAK2 inhibitors (improve spleen size and QoL)

38
Q

Reactive causes of high cell counts are more common than myeloproliferative disorders. True/False?

A

True

39
Q

List some reactive causes of raised granulocytes

A

Infection
Post-surgery
Steroids

40
Q

List some reactive causes of raised platelets

A 
Infection
Iron deficiency
Malignancy
Blood loss
Post-splenectomy
41
Q

List some reactive causes of raised red cells

A

Dehydration
Diuretics
Secondary polycythemia (hypoxia etc.)

y3 haematology (16 decks)

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