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Haematology > Myeloproliferative Neoplasms > Flashcards

Myeloproliferative Neoplasms Flashcards

1
Q

What does myeloproliferative mean?

A 
Myelo = bone marrow lineage 
Proliferative = to grow or multiply by rapidly producing mew tissue
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2
Q

What is a clonal haemopoietic stem cell disorder?

A

Increased production of one or more types of haemopoietic cells

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3
Q

Describe CML

A

Overproduction of granulocytes

BCR-ALB 1 positive = philadelphia chromosome

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4
Q

Describe essential thrombocythaemia?

A

Over-production of platelets

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5
Q

Describe polycythaemia vera

A

Overproduction of red cells

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6
Q

What are the different types of BCR-ABL 1 negative myeloproliferative neoplasms?

A

Primary myelofibrosis
Polycythaemia vera
Essential thrombocythemia

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7
Q

When should a MPN be considered?

A 
High granulocyte count
High red cell count/ Hb
High platelet count 
Eosinophilia/ basophilia 
Splenomegaly 
Thrombosis in an unusual place 
With NO reactive explanation
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8
Q

What are the different phases of CML?

A

Chronic phase
Accelerated phase
Blast crisis

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9
Q

What are the clinical features of CML?

A 
Asymptomatic 
Splenomegaly 
Hypermetabolic symptoms 
Gout 
Misc; problems related to hyperleucocytosis, priapism
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10
Q

What are the lab features of CML?

A

Normal/ decreased Hb

Leucocytosis with neutrophilia and myelocytes, eosinophila, basophilia and thrombocytosis

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11
Q

What is the hallmark of CML?

A

Philadelphia chromosomee

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12
Q

What is the treatment for Philadelphia chromosome positive CML?

A

Tyrosine kinase inhibitor - imatinib

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13
Q

What are features common to MPN?

A

Asymptomatic
Increased cellular turnover (gout, fatigue, wt loss, sweats)
Sy/si due to splenomegaly
Marrow failure; fibrosis or leukaemia transformation; PRV and ET
Thrombosis; TIA, MI, abdo vessel, claudication, erythomelagia

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14
Q

What is polycythaemia vera?

A

High Hb accompanied by erythrocytosis (a true increased in red cells mass) but can have excessive production of other lineages

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15
Q

What do you NEED to distinguish PV from?

A

Secondary polycythaemia; chronic hypoxia, smoking, epo-secreting tumour
Pseudopolycythaemia; dehydration, diuretic therapy, obesity

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16
Q

What are the clinical features of PV?

A

Similar to MPN
Headache, fatigue (blood viscosity raised NOT plasma viscosity)
Itch; aquagenic puritis

17
Q

What are the investigations required for PV?

A

History - investigate for secondary causes (CXR, O2, ABG, drug hx)
Exam; splenomegaly
FBC, film
JAK2 mutation

18
Q

What is the most important test for PV?

A

JAK2 mutation

19
Q

What is JAK2?

A

Kinase

Results in loss of auto-inhibition and activation of epo in the absence of a ligand

20
Q

What is now part of the initial screening in PV?

A

Mutational analysis

21
Q

What is the treatment for PV?

A

Venesect to haematocrit of <0.45
Aspirin
Cytotoxic oral chemo; hydroxycarbamide

22
Q

What is ET?

A

Uncontrolled proliferation of abnormal platelets

Platelet function ABNORMAL; thrombosis, at high levels can cause bleeding due to acquired vWF disease

23
Q

What are the clinical features of ET?

A

Common MPN, particularly vasoocclusive

Bleeding

24
Q

How is ET diagnosed?

A

Exclude reactive thrombocytosis - IMPORTANT

Exclude CML

25
Q

What are the genetics of ET?

A

JAK2 in 50-60%
CALR in 25%
MPL in 5%
10-20% triple neg

26
Q

What is the treatment for ET?

A

Anti-platelets; aspirin

Cytoreductive therapy to control proliferation; hydroxycarbamide, anagrelide or interferon alpha

27
Q

What can cause myelofibrosis?

A

Idiopathic

Post-polycythaemia or essential thrombocythaemia

28
Q

What are the features of idiopathic myelofibrosis?

A 
Marrow failure; variable degrees
Bone marrow fibrosis 
Extramedullary haematopoiesis (liver and spleen) 
Leukoerythroblastic film appearances 
Tear-drop shaped RBC in peripheral blood
29
Q

What are the features of marrow failure?

A

Anaemia
Bleeding
Infection

30
Q

What are the features of splenomegaly?

A

LUQ pain
Cx; portal hypertx
Gastric fullness

31
Q

What are the clinical features of myelofibrosis?

A

Marrow failure
Splenomegaly
Hypercatabolism

32
Q

How is MF diagnosed in the lab?

A

Typical blood film; tear drop shaped RBC and leukoerythroblsatic
Dry aspirate
Fibrosis on trephine biopsy

33
Q

What are causes for a leukoerythroblastic film?

A

Reactive (sepsis)
Marrow infiltration
Myelofibrosis

34
Q

What is a leukoerythroblastic film?

A 
Immature cells (myelocytes and normoblasts) seen on the peripheral blood film
Normoblast = nucleated RBC
35
Q

What is the treatment of MF?

A

Supportive care; blood transfusion, platelets, antibiotics
Allogenic stem cell transplantation
??Splenectomy
JAK2 inhibitors (ruxolitinib)

36
Q

What are the causes for reactive high blood coutns?

A

Reactive granulocytes; infection (pyogenic bacteria causing neutrophilia), physiological (post-surgery, steroids)
Reactive thrombocytosis; infection, IDA, malignancy, blood loss
Reactive polycythaemia; dehydration (pseudopolycythaemia), secondary

Haematology (31 decks)

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